This document discusses hereditary colorectal cancer and polyposis syndromes, specifically familial adenomatous polyposis (FAP). FAP is caused by mutations in the APC gene and is characterized by the development of over 100 adenomatous polyps in the colon by age 20-30. Without treatment, individuals with FAP have a nearly 100% risk of developing colorectal cancer by age 40. Treatment options include medical management with drugs to reduce polyp growth, as well as surgical interventions such as total proctocolectomy with ileostomy, total abdominal colectomy with ileorectal anastomosis, or proctocolectomy with ileal pouch-anal anastomosis
1) Crohn's disease is a chronic inflammatory bowel disease that can affect any part of the gastrointestinal tract. The role of surgery has evolved from being an emergency procedure to one performed after failed medical therapy.
2) The decision to proceed with surgery considers disease classification, complications, risk of recurrence, and side effects of medical therapy. The goals are to resect gross disease while conserving bowel length.
3) Both medical and surgical therapies are important in managing Crohn's disease. Overall treatment requires careful use of both approaches appropriately combined.
This document reports a rare case of inflammatory pseudotumor (IPT) of the stomach in a 65-year-old female patient. IPT is a benign tumor that can occur in any part of the body. The patient presented with abdominal discomfort, constipation, and abdominal distension for a year. Imaging showed a large cystic mass in her stomach. She underwent surgery to remove the mass, which was diagnosed as IPT based on pathological examination. IPT of the stomach is very rare, and this case highlights the difficulty in diagnosis due to non-specific presentations that can mimic other tumors.
This document summarizes an article on cancer of unknown primary origin (CUP). CUP represents about 3% of cancer cases where patients present with metastatic disease but the primary site cannot be identified. Histologically, CUP is usually adenocarcinoma but can also be poorly differentiated carcinoma, squamous cell carcinoma, or other subtypes. Prognosis is generally poor with a median survival of 5-10 months, though some subgroups like women with axillary node involvement only have better outcomes. The document discusses diagnostic approaches and treatment considerations for CUP.
This document discusses hereditary gastrointestinal cancer syndromes. It provides background on genetics versus genomics and compares genetic testing of single genes versus multiple genes simultaneously. Lynch syndrome, also called hereditary nonpolyposis colorectal cancer, is summarized, including the importance of microsatellite instability testing and immunohistochemistry to identify mismatch repair gene mutations. Screening recommendations for Lynch syndrome, such as annual colonoscopy, are discussed to reduce cancer risk.
1. Neuroblastoma is a cancer of the sympathetic nervous system that is most common in children under 5 years old. It ranges from low to high risk based on tumor biology and staging.
2. Diagnosis involves biopsy of the primary tumor followed by imaging to determine staging. The tumor is then classified based on features like cell differentiation and Schwannian stroma content.
3. Presentation depends on tumor location and can include non-specific symptoms like abdominal mass or pain, or syndromes related to nerve involvement like Horner's syndrome.
Yassin M. Alsaleh, a 3-year-old Saudi boy, presented with weight loss, pallor, decreased activity, and fever for 2 weeks. Imaging showed an abdominal mass and bone marrow involvement. Biopsy confirmed stage 4 neuroblastoma with bone marrow metastases. Neuroblastoma is a cancer of the sympathetic nervous system that typically presents in children aged 5 or younger. Risk stratification guides treatment, which may include chemotherapy, surgery, radiation therapy, stem cell transplant, or immunotherapy depending on disease stage, age, genetics, and response to initial therapy. Complications can include cord compression, organ dysfunction, infection, and treatment side effects.
CANCER of UNKNOWN PRIMARY (CUP), Dr BÙI ĐẮC CHÍhungnguyenthien
Cancer of unknown primary (CUP) refers to metastatic cancer that has spread to other parts of the body but the original primary site cannot be identified. CUP makes up approximately 2-6% of cancer cases. Conventional methods to identify the primary site include imaging scans, medical history, physical exams, and laboratory tests. Molecular profiling using techniques like immunohistochemistry, gene expression profiling, and next generation sequencing can identify predictive biomarkers and actionable targets in around 80% of CUP cases. Identifying the primary site can help determine the best treatment approach and predict outcomes. Gene expression profiling tests like the Pathwork Tissue of Origin test can determine the likely tissue of origin with 99% certainty when the highest similarity score is above
Zeine Seminar 2010, Cancer Associated Fibroblasts and Microvascular Prolifera...Rana ZEINE, MD, PhD, MBA
World Cancer Congress 2010
Presence of Cancer-Associated Fibroblasts correlates with Microvascular Proliferation which is a Poor Prognostic Factor in Neuroblastoma Tumors
1) Crohn's disease is a chronic inflammatory bowel disease that can affect any part of the gastrointestinal tract. The role of surgery has evolved from being an emergency procedure to one performed after failed medical therapy.
2) The decision to proceed with surgery considers disease classification, complications, risk of recurrence, and side effects of medical therapy. The goals are to resect gross disease while conserving bowel length.
3) Both medical and surgical therapies are important in managing Crohn's disease. Overall treatment requires careful use of both approaches appropriately combined.
This document reports a rare case of inflammatory pseudotumor (IPT) of the stomach in a 65-year-old female patient. IPT is a benign tumor that can occur in any part of the body. The patient presented with abdominal discomfort, constipation, and abdominal distension for a year. Imaging showed a large cystic mass in her stomach. She underwent surgery to remove the mass, which was diagnosed as IPT based on pathological examination. IPT of the stomach is very rare, and this case highlights the difficulty in diagnosis due to non-specific presentations that can mimic other tumors.
This document summarizes an article on cancer of unknown primary origin (CUP). CUP represents about 3% of cancer cases where patients present with metastatic disease but the primary site cannot be identified. Histologically, CUP is usually adenocarcinoma but can also be poorly differentiated carcinoma, squamous cell carcinoma, or other subtypes. Prognosis is generally poor with a median survival of 5-10 months, though some subgroups like women with axillary node involvement only have better outcomes. The document discusses diagnostic approaches and treatment considerations for CUP.
This document discusses hereditary gastrointestinal cancer syndromes. It provides background on genetics versus genomics and compares genetic testing of single genes versus multiple genes simultaneously. Lynch syndrome, also called hereditary nonpolyposis colorectal cancer, is summarized, including the importance of microsatellite instability testing and immunohistochemistry to identify mismatch repair gene mutations. Screening recommendations for Lynch syndrome, such as annual colonoscopy, are discussed to reduce cancer risk.
1. Neuroblastoma is a cancer of the sympathetic nervous system that is most common in children under 5 years old. It ranges from low to high risk based on tumor biology and staging.
2. Diagnosis involves biopsy of the primary tumor followed by imaging to determine staging. The tumor is then classified based on features like cell differentiation and Schwannian stroma content.
3. Presentation depends on tumor location and can include non-specific symptoms like abdominal mass or pain, or syndromes related to nerve involvement like Horner's syndrome.
Yassin M. Alsaleh, a 3-year-old Saudi boy, presented with weight loss, pallor, decreased activity, and fever for 2 weeks. Imaging showed an abdominal mass and bone marrow involvement. Biopsy confirmed stage 4 neuroblastoma with bone marrow metastases. Neuroblastoma is a cancer of the sympathetic nervous system that typically presents in children aged 5 or younger. Risk stratification guides treatment, which may include chemotherapy, surgery, radiation therapy, stem cell transplant, or immunotherapy depending on disease stage, age, genetics, and response to initial therapy. Complications can include cord compression, organ dysfunction, infection, and treatment side effects.
CANCER of UNKNOWN PRIMARY (CUP), Dr BÙI ĐẮC CHÍhungnguyenthien
Cancer of unknown primary (CUP) refers to metastatic cancer that has spread to other parts of the body but the original primary site cannot be identified. CUP makes up approximately 2-6% of cancer cases. Conventional methods to identify the primary site include imaging scans, medical history, physical exams, and laboratory tests. Molecular profiling using techniques like immunohistochemistry, gene expression profiling, and next generation sequencing can identify predictive biomarkers and actionable targets in around 80% of CUP cases. Identifying the primary site can help determine the best treatment approach and predict outcomes. Gene expression profiling tests like the Pathwork Tissue of Origin test can determine the likely tissue of origin with 99% certainty when the highest similarity score is above
Zeine Seminar 2010, Cancer Associated Fibroblasts and Microvascular Prolifera...Rana ZEINE, MD, PhD, MBA
World Cancer Congress 2010
Presence of Cancer-Associated Fibroblasts correlates with Microvascular Proliferation which is a Poor Prognostic Factor in Neuroblastoma Tumors
Valporic acid for treatment of familial adenomatous polyposismesfin mamuye
Valporic acid is proposed for repurposing to treat Familial adenomatous polyposis (FAP). FAP is characterized by numerous colon polyps and increased cancer risk. Valporic acid may inhibit polyp formation and reduce existing polyp size by inducing histone acetylation and cell growth arrest. It has a favorable safety profile in pediatric brain tumor patients. However, valporic acid has serious potential side effects like hepatotoxicity and birth defects that require careful risk-benefit assessment for its use in FAP.
1. The combination of SMMHC and p63 is ideal for marking myoepithelial cells and assessing features such as stromal invasion and differentiation of in situ versus invasive lesions.
2. Positive 34βE12 and CK5/6 staining within the proliferative component indicates atypical papilloma rather than papilloma with DCIS.
3. Intracystic papillary carcinoma shows positive staining by Collagen IV around ducts, distinguishing it from solid papillary carcinoma.
This document discusses the changing landscape of cancer of unknown primary (CUP) over four decades from 1976 to the present. It describes the evolution from recognition of favorable prognostic subsets in 1976-1986, to improved diagnostic techniques in 1986-1996, to empiric chemotherapy in 1996-2006, and currently to improved pathologic and genetic diagnostic technologies and better outcomes for many CUP patients from 2006 onward. The document provides details on histologic classification, clinicopathologic entities, diagnostic approaches including imaging, histopathology, immunohistochemistry, and molecular analysis, as well as discussion of favorable and unfavorable prognostic subsets and treatment approaches.
This document discusses carcinoma of unknown primary (CUP). Key points:
- CUP is defined as metastatic cancer where the primary site cannot be identified after evaluation. It accounts for 2-5% of cancers and has a median survival of 6-9 months.
- The cancer is often aggressive and may have disseminated before the primary is detectable. Major histologies are adenocarcinoma (60-90%) and squamous cell carcinoma (5%).
- Favorable subsets with better prognosis include peritoneal carcinomatosis resembling ovarian cancer, isolated axillary adenopathy resembling breast cancer, and poorly differentiated neuroendocrine tumors.
- Treatment involves systemic chemotherapy. Selection of regimen depends on
This document provides information on carcinoma of unknown primary (CUP). It defines CUP and discusses the epidemiology, pathology, diagnostic approach, and treatment. Some key points:
- CUP accounts for 2-5% of cancers and has a median survival of 6-9 months. Adenocarcinoma is the most common histology (60%).
- Diagnostic evaluation includes imaging, endoscopy, and immunohistochemistry to identify the primary site. Cytokeratin 7/20 staining patterns provide clues to site of origin.
- Favorable prognostic subsets include isolated axillary adenocarcinoma in women, papillary adenocarcinoma of the peritoneum in women,
Literature Review Of Management Of Pineal Region TumourLiew Boon Seng
- Pineal region tumors make up 0.4-1.0% of intracranial tumors in adults and 3.0-8.0% of brain tumors in children, with most children presenting between ages 10-20 years old.
- MRI with gadolinium is used to evaluate pineal region lesions and assess characteristics like size, vascularity and borders, though tumor type cannot be determined reliably from imaging alone.
- Histopathological examination is needed for diagnosis, as germ cell tumors are the most common in children and include germinomas and other tumors derived from totipotential germ cells.
Neuroblastoma is the most common extracranial solid tumor in children. It arises from neural crest cells that form the adrenal medulla and sympathetic ganglia. Over half of children present with metastatic disease. Risk factors include genetic predispositions and exposure to chemicals during pregnancy. Clinical features vary depending on tumor location but may include abdominal mass, bone pain, or eye symptoms. Treatment involves surgery, chemotherapy, and sometimes radiation. Prognosis depends on age, stage, and biological features of the tumor.
ITC, or isolated tumor cells, refer to small clusters of cancer cells that break off from the primary tumor and circulate in the bloodstream. While only 0.05% of circulating tumor cells survive to form metastases, detection of ITC can provide prognostic information. ITC can be detected using morphological or non-morphological methods like immunohistochemistry and PCR, but non-morphological methods have a higher false positive rate. The presence of ITC may have prognostic value and inform more aggressive treatment, but the evidence for their clinical significance is equivocal for many cancer types. ITC detection is most clearly prognostic in breast cancer, while data is mixed for colorectal cancer.
This document discusses several case scenarios related to neuroblastoma. It begins with the case of a 4-month-old girl brought to the pediatrician for projectile vomiting. An abdominal ultrasound reveals an adrenal mass, and further workup is suggested. The second case involves a 3-year-old boy presenting with hip, leg, and back pain who is found to have a large abdominal mass and decreased breath sounds. The third case is a 4-year-old boy with fever, fatigue, and weight loss, who is found to have a large adrenal mass and widespread lymphadenopathy. Molecular studies confirm MYCN-amplified high-risk neuroblastoma. The document then reviews treatment strategies, complications, and
Neuroblastoma is the third most common childhood cancer and arises from sympathetic nervous system. Staging involves evaluating tumor size, spread and biomarkers. Treatment ranges from observation to intensive chemotherapy and stem cell transplant depending on risk factors like age, stage, genetics and response to initial treatment. Targeted therapies are being studied in recurrent and high risk disease.
- A 45-year-old female patient presented with bone metastases and osteolytic lesions that were diagnosed as adenocarcinoma of unknown primary (CUP) via biopsy of a vertebral lesion.
- CUP accounts for 5% of cancers, where the primary site cannot be identified. Less than 30% are identified before death, though autopsy often reveals the primary site.
- An 18F-FDG PET scan is the most useful test to identify the unknown primary site. Pathological evaluation including immunohistochemistry can determine the tumor type and help narrow the possible primary sites.
This document provides an overview of cancer of unknown primary (CUP). It defines CUP and discusses the epidemiology, pathology, natural history, diagnostic approach, and treatment. Regarding diagnosis, it describes the use of imaging, endoscopy, histopathology including immunohistochemistry, and molecular analysis to identify the primary tumor site. Treatment options are also summarized, noting that platinum-based chemotherapy regimens have response rates of 30-40% and median survival of 8-11 months. Certain favorable prognostic subsets, such as those with specific nodal distributions or tumor markers, may have longer survival.
This document provides an overview of neuroblastoma, including its clinical presentation, pathology, risk grouping, diagnostic workup, prognostic factors, and management. Some key points:
- Neuroblastoma arises from neural crest tissue and is the most common extracranial solid tumor in children. Over 70% present with metastases.
- Risk grouping systems include INSS (International Neuroblastoma Staging System) and more recently INRG (International Neuroblastoma Risk Group) staging.
- Prognostic factors include age, stage, pathology, MYCN status, and other biomarkers.
- Treatment depends on risk but may include surgery, chemotherapy, radiation, stem cell transplant, and immunotherapy. Precise management
An 8 year old boy from Jumla, Nepal presented with increased urinary frequency and was found to have a 4cmx4cm mass in his abdomen. Imaging and biopsy revealed the mass was a stage III small round cell tumor. Further tests showed it was a neuroblastoma that had metastasized to his bones, classifying it as stage IV disease. Neuroblastoma is the third most common childhood cancer, arising from sympathetic nervous tissue. Prognosis depends on age at diagnosis, tumor stage, genetics, and response to chemotherapy and surgery.
This is the clinical case of a 75-year-old male patient with medical history of Stage III-A immunoglobulin G kappa multiple myeloma with advanced bone disease, who presented right testicular tumor 1 month ago. Scrotal ultrasound showed an heterogeneous 35 × 22 × 52 mm right testis with decreased echogenicity and increased vascularization corresponding to testicular tumor. Tumor markers were normal and computed tomography scan showed no extension of disease. Pathologist confirmed with immunohistochemical study the definitive diagnosis of testicular plasmacytoma. The patient started the third cycle of treatment with monoclonal antibodies, with good initial response.
Inter group rhabdomyosarcoma study group (irsg)Ajay Manickam
1. The IRSG study from 1972-1997 evaluated treatment protocols for rhabdomyosarcoma and established a staging and grouping system to determine prognosis and guide therapy.
2. Results showed surgery is important, with Group I having the best outlook while Group IV has the worst. Radiation therapy improved outcomes for Groups II and III. Chemotherapy including VAC or VIE improved failure-free and overall survival.
3. IRSG V recommendations incorporate risk stratification to allow for multidisciplinary treatment tailored to histology, with the goal of local control while preserving function.
This study aimed to evaluate the role of delayed primary excision (DPE) surgery after induction chemotherapy in localized nonmetastatic adult head and neck rhabdomyosarcoma. The study retrospectively analyzed 24 patients treated with either DPE followed by surgery, radiotherapy and chemotherapy or chemoradiotherapy without surgery. It found that 59% of DPE patients had a complete resection but 82% had residual viable tumors. Response to chemotherapy significantly impacted local control, with 100% local control for good responders versus 33% for poor responders. In good responders, DPE resulted in significantly better local control than chemoradiotherapy alone. However, treatment modality did not impact overall survival. Distant metastases
Principles of medical_oncology dr. varunVarun Goel
- The document discusses several key principles of medical oncology including that cancer treatment is multidisciplinary, early stage cancers are more curable than late stage, and the best treatment is often found in clinical trials.
- It describes the basic tenets of chemotherapy including that it can be used for induction treatment of advanced cancers or as adjuvant treatment after local therapy to treat high risk of recurrence. The intent of chemotherapy can be curative or palliative.
- Several models of tumor growth and response to chemotherapy are explained including the Skipper-Wilcox model, concepts of combination chemotherapy, and the Goldie-Coldman model regarding emergence of drug resistance with increased tumor size.
Colonic polyposis refers to numerous polyps throughout the GI tract that are often precancerous. The most common type is familial adenomatous polyposis (FAP), an autosomal dominant condition caused by a mutation in the APC gene. People with FAP develop hundreds to thousands of colon polyps by their mid-30s, and colon cancer is inevitable without surgery to remove the colon. They are also at risk of polyps in the stomach and duodenum that can become cancerous. Treatment involves prophylactic colectomy, surveillance of the upper GI tract, and managing extracolonic manifestations such as osteomas and desmoid tumors.
This document discusses various types of colorectal polyps and polyposis syndromes. It begins by defining different types of colorectal polyps based on size, attachment, cellular architecture, and histological appearance. Larger polyps have a higher likelihood of harboring cancer. The main polyposis syndromes discussed are familial adenomatous polyposis (FAP), hereditary non-polyposis colorectal cancer (HNPCC), Peutz-Jeghers syndrome, and juvenile polyposis syndrome. FAP is characterized by hundreds of colonic polyps and a 100% risk of colon cancer. Management involves prophylactic colectomy and surveillance of other organs for extracol
Valporic acid for treatment of familial adenomatous polyposismesfin mamuye
Valporic acid is proposed for repurposing to treat Familial adenomatous polyposis (FAP). FAP is characterized by numerous colon polyps and increased cancer risk. Valporic acid may inhibit polyp formation and reduce existing polyp size by inducing histone acetylation and cell growth arrest. It has a favorable safety profile in pediatric brain tumor patients. However, valporic acid has serious potential side effects like hepatotoxicity and birth defects that require careful risk-benefit assessment for its use in FAP.
1. The combination of SMMHC and p63 is ideal for marking myoepithelial cells and assessing features such as stromal invasion and differentiation of in situ versus invasive lesions.
2. Positive 34βE12 and CK5/6 staining within the proliferative component indicates atypical papilloma rather than papilloma with DCIS.
3. Intracystic papillary carcinoma shows positive staining by Collagen IV around ducts, distinguishing it from solid papillary carcinoma.
This document discusses the changing landscape of cancer of unknown primary (CUP) over four decades from 1976 to the present. It describes the evolution from recognition of favorable prognostic subsets in 1976-1986, to improved diagnostic techniques in 1986-1996, to empiric chemotherapy in 1996-2006, and currently to improved pathologic and genetic diagnostic technologies and better outcomes for many CUP patients from 2006 onward. The document provides details on histologic classification, clinicopathologic entities, diagnostic approaches including imaging, histopathology, immunohistochemistry, and molecular analysis, as well as discussion of favorable and unfavorable prognostic subsets and treatment approaches.
This document discusses carcinoma of unknown primary (CUP). Key points:
- CUP is defined as metastatic cancer where the primary site cannot be identified after evaluation. It accounts for 2-5% of cancers and has a median survival of 6-9 months.
- The cancer is often aggressive and may have disseminated before the primary is detectable. Major histologies are adenocarcinoma (60-90%) and squamous cell carcinoma (5%).
- Favorable subsets with better prognosis include peritoneal carcinomatosis resembling ovarian cancer, isolated axillary adenopathy resembling breast cancer, and poorly differentiated neuroendocrine tumors.
- Treatment involves systemic chemotherapy. Selection of regimen depends on
This document provides information on carcinoma of unknown primary (CUP). It defines CUP and discusses the epidemiology, pathology, diagnostic approach, and treatment. Some key points:
- CUP accounts for 2-5% of cancers and has a median survival of 6-9 months. Adenocarcinoma is the most common histology (60%).
- Diagnostic evaluation includes imaging, endoscopy, and immunohistochemistry to identify the primary site. Cytokeratin 7/20 staining patterns provide clues to site of origin.
- Favorable prognostic subsets include isolated axillary adenocarcinoma in women, papillary adenocarcinoma of the peritoneum in women,
Literature Review Of Management Of Pineal Region TumourLiew Boon Seng
- Pineal region tumors make up 0.4-1.0% of intracranial tumors in adults and 3.0-8.0% of brain tumors in children, with most children presenting between ages 10-20 years old.
- MRI with gadolinium is used to evaluate pineal region lesions and assess characteristics like size, vascularity and borders, though tumor type cannot be determined reliably from imaging alone.
- Histopathological examination is needed for diagnosis, as germ cell tumors are the most common in children and include germinomas and other tumors derived from totipotential germ cells.
Neuroblastoma is the most common extracranial solid tumor in children. It arises from neural crest cells that form the adrenal medulla and sympathetic ganglia. Over half of children present with metastatic disease. Risk factors include genetic predispositions and exposure to chemicals during pregnancy. Clinical features vary depending on tumor location but may include abdominal mass, bone pain, or eye symptoms. Treatment involves surgery, chemotherapy, and sometimes radiation. Prognosis depends on age, stage, and biological features of the tumor.
ITC, or isolated tumor cells, refer to small clusters of cancer cells that break off from the primary tumor and circulate in the bloodstream. While only 0.05% of circulating tumor cells survive to form metastases, detection of ITC can provide prognostic information. ITC can be detected using morphological or non-morphological methods like immunohistochemistry and PCR, but non-morphological methods have a higher false positive rate. The presence of ITC may have prognostic value and inform more aggressive treatment, but the evidence for their clinical significance is equivocal for many cancer types. ITC detection is most clearly prognostic in breast cancer, while data is mixed for colorectal cancer.
This document discusses several case scenarios related to neuroblastoma. It begins with the case of a 4-month-old girl brought to the pediatrician for projectile vomiting. An abdominal ultrasound reveals an adrenal mass, and further workup is suggested. The second case involves a 3-year-old boy presenting with hip, leg, and back pain who is found to have a large abdominal mass and decreased breath sounds. The third case is a 4-year-old boy with fever, fatigue, and weight loss, who is found to have a large adrenal mass and widespread lymphadenopathy. Molecular studies confirm MYCN-amplified high-risk neuroblastoma. The document then reviews treatment strategies, complications, and
Neuroblastoma is the third most common childhood cancer and arises from sympathetic nervous system. Staging involves evaluating tumor size, spread and biomarkers. Treatment ranges from observation to intensive chemotherapy and stem cell transplant depending on risk factors like age, stage, genetics and response to initial treatment. Targeted therapies are being studied in recurrent and high risk disease.
- A 45-year-old female patient presented with bone metastases and osteolytic lesions that were diagnosed as adenocarcinoma of unknown primary (CUP) via biopsy of a vertebral lesion.
- CUP accounts for 5% of cancers, where the primary site cannot be identified. Less than 30% are identified before death, though autopsy often reveals the primary site.
- An 18F-FDG PET scan is the most useful test to identify the unknown primary site. Pathological evaluation including immunohistochemistry can determine the tumor type and help narrow the possible primary sites.
This document provides an overview of cancer of unknown primary (CUP). It defines CUP and discusses the epidemiology, pathology, natural history, diagnostic approach, and treatment. Regarding diagnosis, it describes the use of imaging, endoscopy, histopathology including immunohistochemistry, and molecular analysis to identify the primary tumor site. Treatment options are also summarized, noting that platinum-based chemotherapy regimens have response rates of 30-40% and median survival of 8-11 months. Certain favorable prognostic subsets, such as those with specific nodal distributions or tumor markers, may have longer survival.
This document provides an overview of neuroblastoma, including its clinical presentation, pathology, risk grouping, diagnostic workup, prognostic factors, and management. Some key points:
- Neuroblastoma arises from neural crest tissue and is the most common extracranial solid tumor in children. Over 70% present with metastases.
- Risk grouping systems include INSS (International Neuroblastoma Staging System) and more recently INRG (International Neuroblastoma Risk Group) staging.
- Prognostic factors include age, stage, pathology, MYCN status, and other biomarkers.
- Treatment depends on risk but may include surgery, chemotherapy, radiation, stem cell transplant, and immunotherapy. Precise management
An 8 year old boy from Jumla, Nepal presented with increased urinary frequency and was found to have a 4cmx4cm mass in his abdomen. Imaging and biopsy revealed the mass was a stage III small round cell tumor. Further tests showed it was a neuroblastoma that had metastasized to his bones, classifying it as stage IV disease. Neuroblastoma is the third most common childhood cancer, arising from sympathetic nervous tissue. Prognosis depends on age at diagnosis, tumor stage, genetics, and response to chemotherapy and surgery.
This is the clinical case of a 75-year-old male patient with medical history of Stage III-A immunoglobulin G kappa multiple myeloma with advanced bone disease, who presented right testicular tumor 1 month ago. Scrotal ultrasound showed an heterogeneous 35 × 22 × 52 mm right testis with decreased echogenicity and increased vascularization corresponding to testicular tumor. Tumor markers were normal and computed tomography scan showed no extension of disease. Pathologist confirmed with immunohistochemical study the definitive diagnosis of testicular plasmacytoma. The patient started the third cycle of treatment with monoclonal antibodies, with good initial response.
Inter group rhabdomyosarcoma study group (irsg)Ajay Manickam
1. The IRSG study from 1972-1997 evaluated treatment protocols for rhabdomyosarcoma and established a staging and grouping system to determine prognosis and guide therapy.
2. Results showed surgery is important, with Group I having the best outlook while Group IV has the worst. Radiation therapy improved outcomes for Groups II and III. Chemotherapy including VAC or VIE improved failure-free and overall survival.
3. IRSG V recommendations incorporate risk stratification to allow for multidisciplinary treatment tailored to histology, with the goal of local control while preserving function.
This study aimed to evaluate the role of delayed primary excision (DPE) surgery after induction chemotherapy in localized nonmetastatic adult head and neck rhabdomyosarcoma. The study retrospectively analyzed 24 patients treated with either DPE followed by surgery, radiotherapy and chemotherapy or chemoradiotherapy without surgery. It found that 59% of DPE patients had a complete resection but 82% had residual viable tumors. Response to chemotherapy significantly impacted local control, with 100% local control for good responders versus 33% for poor responders. In good responders, DPE resulted in significantly better local control than chemoradiotherapy alone. However, treatment modality did not impact overall survival. Distant metastases
Principles of medical_oncology dr. varunVarun Goel
- The document discusses several key principles of medical oncology including that cancer treatment is multidisciplinary, early stage cancers are more curable than late stage, and the best treatment is often found in clinical trials.
- It describes the basic tenets of chemotherapy including that it can be used for induction treatment of advanced cancers or as adjuvant treatment after local therapy to treat high risk of recurrence. The intent of chemotherapy can be curative or palliative.
- Several models of tumor growth and response to chemotherapy are explained including the Skipper-Wilcox model, concepts of combination chemotherapy, and the Goldie-Coldman model regarding emergence of drug resistance with increased tumor size.
Colonic polyposis refers to numerous polyps throughout the GI tract that are often precancerous. The most common type is familial adenomatous polyposis (FAP), an autosomal dominant condition caused by a mutation in the APC gene. People with FAP develop hundreds to thousands of colon polyps by their mid-30s, and colon cancer is inevitable without surgery to remove the colon. They are also at risk of polyps in the stomach and duodenum that can become cancerous. Treatment involves prophylactic colectomy, surveillance of the upper GI tract, and managing extracolonic manifestations such as osteomas and desmoid tumors.
This document discusses various types of colorectal polyps and polyposis syndromes. It begins by defining different types of colorectal polyps based on size, attachment, cellular architecture, and histological appearance. Larger polyps have a higher likelihood of harboring cancer. The main polyposis syndromes discussed are familial adenomatous polyposis (FAP), hereditary non-polyposis colorectal cancer (HNPCC), Peutz-Jeghers syndrome, and juvenile polyposis syndrome. FAP is characterized by hundreds of colonic polyps and a 100% risk of colon cancer. Management involves prophylactic colectomy and surveillance of other organs for extracol
This document discusses colorectal cancer (CRC) and its precursors and hereditary syndromes. It covers topics like adenomatous polyps, genetic mutations involved in CRC development, hereditary CRC syndromes like familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC), risk factors, screening methods, and prevention strategies. Colonoscopy is recommended as the preferred screening method as it allows both diagnosis and removal of adenomas.
1. Colorectal cancer develops from adenomatous polyps over many years through the accumulation of genetic mutations in tumor suppressor genes, oncogenes, and DNA repair mechanisms.
2. The major hereditary colorectal cancer syndromes are familial adenomatous polyposis and hereditary nonpolyposis colorectal cancer, which are associated with mutations in the APC and DNA MMR genes respectively.
3. Patients with inflammatory bowel disease or a family history of colorectal cancer are at increased risk of developing colorectal cancer.
Familial adenomatous polyposis (FAP) is an inherited condition characterized by the development of hundreds of colon polyps in early adulthood, which inevitably leads to colon cancer if left untreated. It is caused by a mutation in the APC gene and is inherited in an autosomal dominant pattern. Affected individuals require total colectomy to remove the colon before cancer develops, as well as lifelong surveillance of the rectum if it is spared, and other organs like the stomach and small intestine where polyps and cancer can also occur. Screening and management aims to detect and treat polyps and cancers early to improve outcomes for individuals and their at-risk family members.
Colorectal cancer results from the accumulation of genetic mutations that progress from normal tissue to dysplastic adenomas to carcinoma over approximately 15 years. Patients with certain hereditary syndromes like familial adenomatous polyposis and hereditary non-polyposis colorectal cancer are at very high risk and require aggressive screening. Screening options for average risk patients include fecal occult blood testing, flexible sigmoidoscopy, and colonoscopy which can detect and remove precancerous polyps, reducing colorectal cancer incidence and mortality.
1. Colorectal cancer develops from adenomatous polyps through a series of genetic mutations over many years. Only about 1 in 20 polyps progress to cancer.
2. The two major hereditary colorectal cancer syndromes are familial adenomatous polyposis and hereditary nonpolyposis colorectal cancer. People with a family history of colorectal cancer or polyps are also at higher risk.
3. Screening tests like colonoscopy can detect and remove polyps, preventing colorectal cancer. Regular screening is recommended for those over 50 or with a family history of colorectal cancer.
Colon ca. , presentation , pathophysiology , and treatmentIbrahimAlbujays
This document discusses colon cancer, including risk factors, pathogenesis, types of polyps, staging, and treatment. The main risk factors for colon cancer are age, personal history of colon cancer or inflammatory bowel disease, smoking, and obesity. Genetic defects like in the APC gene can cause familial adenomatous polyposis (FAP). Colon cancer is typically staged after diagnosis and treatment may involve surgery like colectomy along with chemotherapy and follow-up surveillance colonoscopies.
Colorectal carcinoma is the fourth most common cancer in males and third most common in females. It arises from the transformation of colorectal epithelial cells through a multi-step process involving mutations in genes like APC, KRAS, and p53. Pathological examination provides information on tumor stage, grade, and molecular characteristics that influence prognosis and guide treatment decisions. Molecular testing can also identify hereditary syndromes associated with colorectal cancer risk.
This document discusses ovarian tumors, specifically germ cell tumors. It provides information on the classification, histopathology, immunoprofile, and other characteristics of various types of ovarian germ cell tumors including dysgerminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, immature teratoma, and mature cystic teratoma. It also mentions other rare subtypes such as struma ovarii. The document aims to provide pathology residents with comprehensive information on diagnosing and classifying these tumor types.
Colon cancer epidemiology, risk factors, and etiology, pathology, screening, diagnosis, workup, staging, treatment, chemotherapy and follow-up.
These slides are selections from the major references in surgery, oncology, and internal medicine. I have tried to gather the information from valid and recently-updated references such as NCCN guidelines and Cancer statistics. I hope it helps!
MULTIPROBE FLUORESENCE IN SITU HYBRIDISATION For Detection Of.pptxDrmustafa Ali
Urinary cytology is useful for detecting high-grade urothelial carcinoma but has low sensitivity for low-grade carcinoma. Fluorescence in situ hybridization (FISH) aids in detecting chromosomal abnormalities associated with urothelial carcinoma like gains on chromosomes 3, 7 and 17 and deletions on chromosome 9p21. FISH analysis of urine samples provides higher sensitivity and specificity than cytology alone, especially for low-grade carcinoma and carcinoma of the upper urinary tract. It can help manage cases with atypical cytology and monitor patients after BCG treatment when cytology results are difficult to interpret.
Tumour Markers are substances present in the tumour, produced by the tumour or by the host as a response to the presence of the tumour, providing information about biological characteristics of the tumour. these tumour markers may specific for the tissue but often get elevated in neoplastic as well non-neoplastic lesions, further Various analytical platforms available for serum tumour markers lack standardisation. These factors add to interpretative challenges in serum tumour markers
This document discusses neck dissection procedures for treating cervical lymph node metastases. It begins by outlining factors that influence the incidence of neck metastases from various head and neck cancers. These include the size and characteristics of the primary tumor. It then discusses staging of neck cancer and limitations of current staging systems. The indications for neck dissection are described as either therapeutic for palpable disease or elective for occult metastases. Comprehensive neck dissections remove lymph nodes from levels I-VI, while selective dissections remove only nodes at specific levels predicted to contain metastases from the primary site.
Malignant ascites, an abnormal accumulation of fluid in the abdominal cavity, is commonly associated with cancers like ovarian cancer, gastrointestinal cancers, and breast cancer. It develops due to mechanical obstruction of lymphatic drainage by tumors and increased vascular permeability caused by cytokines. Diagnosis involves abdominal ultrasound or CT scan followed by diagnostic paracentesis of the fluid to examine for malignant cells. Treatment options include dietary salt restriction, diuretics, repeated paracentesis, indwelling catheters, peritoneovenous shunting, and intraperitoneal chemotherapy.
Gallbladder cancer is an uncommon but highly fatal cancer. It is most often diagnosed in patients undergoing surgery for gallbladder stones. Over 90% of gallbladder cancer patients have gallstones or chronic gallbladder inflammation. Major risk factors include female sex, older age, obesity, smoking, and gallbladder diseases. Symptoms are often vague, and many cases are diagnosed late when the cancer has spread. Surgery offers the best chance of treatment when the cancer is localized, but palliative options are usually necessary for advanced cases. Prognosis remains very poor due to late diagnosis and limited effective therapies.
This document summarizes rare and unusual types of urological cancers. It discusses rare subtypes of renal cell carcinoma defined by their histology and genetic abnormalities. It also describes rare histological variants of bladder cancer and non-urothelial cancers of the bladder such as squamous cell carcinoma, adenocarcinoma, small cell carcinoma, and lymphomas. Finally, it briefly outlines other rare non-urothelial cancers including sarcomas, carcinoid tumors, and other even less common malignancies of the bladder.
The document discusses carcinoma of the pancreas. It covers the anatomy, epidemiology, risk factors, genetics, screening, staging, pathologic conditions, clinical presentation, evaluation, management approaches for resectable, borderline resectable, and unresectable disease, surgical procedures including Whipple procedure and distal pancreatectomy, complications, adjuvant therapy approaches studied in trials such as ESPAC-1, and the role of chemoradiation following gemcitabine chemotherapy.
Similar to Acs0514 Hereditary Colorectal Cancer And Polyposis Syndromes 2005 (20)
Acs0522 procedures for benign and malignant biliary tract disease-2005medbookonline
This document discusses procedures for benign and malignant biliary tract diseases. It provides guidance on preoperative evaluation and management of biliary obstruction. Specific considerations are given to infection, renal dysfunction, impaired immunity, malnutrition, and coagulation issues. The document outlines operative planning details such as patient positioning, exposure techniques, and guidelines for biliary anastomoses including suture placement and techniques for difficult access situations.
This document discusses the anatomy and surgical procedure of splenectomy. It describes:
- The spleen's highly variable arterial blood supply, which can take bundled or distributed patterns. This variability impacts the difficulty of surgery.
- The splenic artery typically branches off the celiac axis but can originate from other nearby arteries in rare cases.
- Additional branches of the splenic artery before it enters the spleen, including short gastric and pancreatic arteries.
- A history of splenectomy beginning in the 16th century and its increasing use through the 20th century for trauma and hematologic disorders.
- The development of laparoscopic splenectomy in the early 1990s and ongoing refinement of minim
Gastrostomy is commonly used as a temporary procedure to avoid discomfort from prolonged nasogastric suction after major abdominal surgery. It can also be used permanently when the esophagus is obstructed to nonresectable cancer. The Stamm gastrostomy is most common temporary procedure where a catheter is placed through the stomach wall and anchored to the skin. The Janeway gastrostomy is a permanent alternative where a flap of stomach is brought through the abdominal wall and attached to form a mucosal lined tube to prevent regurgitation. Postoperative care involves gradual advancement to oral intake as the stomach heals and functions return to normal.
This document describes the Billroth I gastric resection procedure, which involves removing part of the stomach and reattaching it to the duodenum. Key steps include transecting the stomach, attaching it to the duodenum using a circular stapler, and closing the gastrotomy site. The procedure aims to control peptic ulcers by combining hemigastrectomy with vagotomy while restoring normal gastrointestinal continuity. Postoperative care focuses on gradual advancement of oral intake and monitoring for complications.
This document describes the Billroth I procedure for gastroduodenostomy. It involves extensive mobilization of the stomach and duodenum to allow for an end-to-end anastomosis between the stomach and duodenum, restoring normal continuity of the gastrointestinal tract. The stomach is divided and sutured closed, then sutured to the duodenum in layers to create the gastroduodenal connection. Postoperative care focuses on gradual advancement of diet and monitoring for gastric retention to support healing and prevent complications.
Gastrostomy is commonly used as a temporary procedure to avoid discomfort from prolonged nasogastric suction after major abdominal surgery. It can also be used permanently when the esophagus is obstructed to nonresectable cancer. The Stamm gastrostomy is most common temporary procedure where a catheter is placed through the stomach wall and anchored to prevent leakage. The Janeway gastrostomy is a permanent alternative where a flap of stomach is brought through the abdominal wall and lined with mucosa to form a permanent opening, preventing regurgitation. Postoperative care involves gradual advancement to oral intake as the stomach and bowel recover function.
Gastrojejunostomy is a surgical procedure that connects the stomach directly to the jejunum. It is indicated for patients with duodenal ulcers complicated by pyloric obstruction or nonresectable stomach or pancreatic cancers causing obstruction. The procedure involves opening the stomach and jejunum, suturing them together to form a stoma, then closing in multiple layers. Postoperatively, gastric emptying is monitored and diet advanced gradually to ensure proper healing.
This document provides guidance on treating a perforated ulcer or subphrenic abscess. It describes:
1) Preparing patients preoperatively by administering IV fluids/antibiotics and gastric suction.
2) Closing perforations by suturing the ulcer and reinforcing it with omentum, or sealing it if too indurated.
3) Draining subphrenic abscesses extraperitoneally by making incisions below the costal margin or through the 12th rib bed and inserting drains into the abscess cavity.
A C S0103 Perioperative Considerations For Anesthesiamedbookonline
This document discusses perioperative considerations for anesthesia. It notes advancements in modern surgical care and alterations in anesthetic management to maximize patient benefit. A preoperative evaluation is important to assess medical history and current medications. Certain medications may need to be adjusted or discontinued before surgery, such as MAOIs, oral anticoagulants, and some herbal supplements, to reduce risks of adverse reactions or bleeding complications during the procedure. The risks and options for anesthesia should be discussed with the patient.
A C S0105 Postoperative Management Of The Hospitalized Patientmedbookonline
This document discusses postoperative management of surgical patients. It describes the different levels of postoperative care including same-day surgery, the surgical floor, telemetry ward, and intensive care unit. Factors determining a patient's disposition include their preoperative health, procedure performed, and postoperative clinical status. The document also discusses common postoperative orders related to tubes, drains, oxygen therapy, and wound care to guide nursing staff.
Postoperative pain is a complex experience involving sensory, emotional, and mental components. Effective pain management is important for patient comfort and recovery. Guidelines for postoperative pain treatment have been developed for specific procedures. Multimodal analgesic regimens targeting multiple pathways are recommended over reliance on opioids alone to prevent tolerance and hyperalgesia. Nonpharmacological complementary therapies can be combined with drug treatments to enhance pain control.
The document discusses the approach to a patient experiencing ongoing bleeding. It outlines the following key steps:
1. First consider the possibility of a technical cause like an unligated vessel and examine for injuries.
2. If no technical cause is found, check the patient's temperature and perform laboratory tests. Hypothermia can cause coagulopathy.
3. Evaluate test results along with the patient's history for clues to underlying causes like platelet dysfunction, coagulation factor deficiencies, or inherited bleeding disorders. Treat the specific condition while continuing evaluation.
A C S0812 Brain Failure And Brain Deathmedbookonline
This document discusses brain failure and brain death. It defines different levels of impaired consciousness from cloudy consciousness to coma. It describes how brain failure results from cardiac arrest and the challenges of restarting the brain after lack of oxygen. It outlines the criteria for diagnosing brain death, including absence of brain stem reflexes and apnea testing. It also discusses the evolution of determining death as technology has allowed life support to prolong vital signs indefinitely.
This document summarizes key points about surgical treatment of early rectal cancer and care of elderly surgical patients. It discusses that radical resection for early rectal cancer achieves excellent local control but has risks, while local excision may be preferable but has a higher local recurrence rate. Adjuvant therapy after local excision may help address this. It also notes that the elderly population is growing and physiologic changes with aging, like cardiac function decline, must be considered in surgical planning and risk assessment for elderly patients. Functional status is more important than age alone.
This document provides information on parotidectomy surgery and the Fundamentals of Laparoscopic Surgery (FLS) program.
It describes the technique for parotidectomy surgery, including identifying and dissecting around the facial nerve. It notes that most parotid tumors are benign and complications are usually temporary facial nerve paralysis.
It then discusses the development of the FLS program to standardize laparoscopic surgery training. The program includes cognitive training and manual skills assessment. Many residency programs and hospitals now require surgeons to complete the FLS. A large grant will help make the program more accessible to residency programs.
This document summarizes an article about volunteer surgeons providing care to wounded soldiers in Iraq and Afghanistan. It discusses the senior visiting surgeon program established by the American College of Surgeons that allows surgeons to volunteer their time. The volunteer rotation described involved caring for patients at Landstuhl Regional Medical Center in Germany as part of the complex medical evacuation process bringing wounded soldiers from war zones to the United States for further treatment and recovery.
1. The document discusses various sources of data for benchmarking surgical outcomes, including public reporting programs, public use administrative databases, and clinical registries. It notes limitations of using administrative data including problems with accuracy, completeness, and clinical precision of coding.
2. Clinical registries like the National Surgical Quality Improvement Program (NSQIP) and the Society of Thoracic Surgeons database are described as better sources of benchmarking data as they provide risk-adjusted outcomes while protecting individual hospital and surgeon confidentiality.
3. Limitations of all surgical benchmarking sources include small sample sizes, lack of generalizability between databases, and lack of external auditing to ensure accuracy and completeness of submitted data.
This document discusses organ procurement from cadaveric donors. It describes the coordination between donor and recipient activities, including matching organs to recipients based on factors like blood type, medical urgency, and waiting time. The evaluation of donor organs is outlined for different organs. Careful donor management aims to optimize organs while respecting donor dignity.
Hand-assisted laparoscopic surgery (HALS) is a hybrid technique that provides many of the advantages of traditional open surgery and laparoscopic colectomy. HALS employs a special access device that allows the surgeon to place a hand in the abdomen to assist with retraction, dissection, and visualization while maintaining pneumoperitoneum and laparoscopic instrumentation through trocars. Studies have shown HALS results in shorter operative times and lower conversion rates to open surgery compared to traditional laparoscopic colectomy while preserving similar short-term clinical outcomes. HALS may help expand the use of minimally invasive approaches for complex colectomies by providing an easier transition from open surgery than traditional laparoscopic techniques.
The document summarizes the evolution of trauma surgery training and practice in the United States. It discusses how trauma surgery originated in large city hospitals but has since expanded to regional trauma centers. It also notes changes in surgical training away from generalist models towards increased specialization. Trauma surgery is increasingly encompassing broader emergency general surgery duties due to workforce shortages, while training programs emphasize specialized rather than broad skills.