Multiple endocrine neoplasia (MEN) type 1 is characterized by tumors of the parathyroid glands, pancreas, and pituitary gland. It is caused by mutations in the MEN1 gene which codes for the menin protein. Patients with MEN1 often present with hyperparathyroidism, pancreatic neuroendocrine tumors, or prolactin-secreting pituitary adenomas. Diagnosis involves blood tests and imaging to identify affected organs. Treatment depends on the specific tumors but may include parathyroidectomy, surgical removal of pancreatic tumors, and medication for functional tumors. Ongoing screening is important due to the risk of additional tumors developing over time.
multiple myloma
By: Nader Amir Al-assadi
Supervised by : Dr/ Ghazi Alariqe
taiz university
Multiple myeloma (MM) is a plasma cell malignancy in which monoclonal plasma cells proliferate in bone marrow, resulting in an over abundance of monoclonal para protein (M protein), destruction of bone, and displacement of other hematopoietic cell lines.
The precise etiology of MM has not yet been established.
Roles have been suggested for a variety of factors, including genetic causes, environmental or occupational causes,radiation, chronic inflammation, and infection .
Acute scrotum is a general term referring to an emergency condition affecting the contents or the wall of the scrotum.
There are a number of conditions that present acutely, predominantly with pain and/or swelling
A careful and detailed history and examination, and in some cases, investigations allow differentiation between these diagnoses. A prompt diagnosis is essential as the patient may require urgent surgical intervention
Testicular torsion refers to twisting of the spermatic cord, causing ischaemia of the testicle.
Testicular torsion results from inadequate fixation of the testis to the tunica vaginalis producing ischemia from reduced arterial inflow and venous outflow obstruction.
The prevalence of testicular torsion in adult patients hospitalized with acute scrotal pain is approximately 25 to 50 percent
Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
Ethanol (CH3CH2OH), or beverage alcohol, is a two-carbon alcohol
that is rapidly distributed in the body and brain. Ethanol alters many
neurochemical systems and has rewarding and addictive properties. It
is the oldest recreational drug and likely contributes to more morbidity,
mortality, and public health costs than all illicit drugs combined. The
5th edition of the Diagnostic and Statistical Manual of Mental Disorders
(DSM-5) integrates alcohol abuse and alcohol dependence into a single
disorder called alcohol use disorder (AUD), with mild, moderate,
and severe subclassifications (American Psychiatric Association, 2013).
In the DSM-5, all types of substance abuse and dependence have been
combined into a single substance use disorder (SUD) on a continuum
from mild to severe. A diagnosis of AUD requires that at least two of
the 11 DSM-5 behaviors be present within a 12-month period (mild
AUD: 2–3 criteria; moderate AUD: 4–5 criteria; severe AUD: 6–11 criteria).
The four main behavioral effects of AUD are impaired control over
drinking, negative social consequences, risky use, and altered physiological
effects (tolerance, withdrawal). This chapter presents an overview
of the prevalence and harmful consequences of AUD in the U.S.,
the systemic nature of the disease, neurocircuitry and stages of AUD,
comorbidities, fetal alcohol spectrum disorders, genetic risk factors, and
pharmacotherapies for AUD.
Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
NVBDCP.pptx Nation vector borne disease control programSapna Thakur
NVBDCP was launched in 2003-2004 . Vector-Borne Disease: Disease that results from an infection transmitted to humans and other animals by blood-feeding arthropods, such as mosquitoes, ticks, and fleas. Examples of vector-borne diseases include Dengue fever, West Nile Virus, Lyme disease, and malaria.
2. Background
Multiple endocrine neoplasia (MEN) syndromes consist
of 2 categories, MEN type 1 (MEN1) and MEN type 2
(MEN2). MEN2 has been subcategorized into MEN2-
A, MEN2-B, and medullary thyroid cancer (MTC)–
only.
3.
4. MEN type 1
The combination of parathyroid tumors, pancreatic
islet cell tumors, and anterior pituitary tumors is
characteristic of MEN1.
usually inherited as an autosomal dominant disorder
can also occur sporadically (without a family history)
In 1954, Wermer was the first to describe the syndrome
as a distinct clinical entity.
5.
6.
7. Etiology
The responsible gene for (MEN1) is located at 11q13 and
codes for the menin protein. Menin is involved with
regulation of transcription and genome stability. Loss
of heterozygosity for this region is associated with
MEN1, suggesting that the gene has tumor suppression
function.
MEN1 is an autosomal dominant disorder, but sporadic
mutations also occur.
8. Pathophysiology and presentations
Parathyroid tumors
Primary hyperparathyroidism, caused by hyperplasia of
parathyroid glands, is the most common manifestation of
multiple endocrine neoplasia type 1 (MEN1) and occurs in
approximately 90% of all patients.
Clinical manifestations can include hypercalcemia,
nephrolithiasis, and bone abnormalities (osteitis fibrosa
cystica).
Common symptoms associated with hypercalcemia include
polydipsia, polyuria, constipation, and generalized malaise.
9. Pancreatic islet cell tumors (neuroendocrine tumors)
the second most common manifestation of MEN1 30-80% of
patients.
Islet cell tumors encompass gastrinomas, insulinomas,
glucagonomas ,vasoactive intestinal polypeptidomas
(VIPomas), and pancreatic polypeptidomas (PPomas).
often multicentric and may undergo malignant
transformation. These tumors can produce peptides and
biogenic amines.
However, nonfunctioning pancreatic endocrine tumors are
the most common enteropancreatic neuroendocrine tumor.
important to diagnose, as malignant pancreatic
neuroendocrine tumors are the most frequent cause of
death in people with MEN1.
10. Gastrinomas
the most common functional pancreatic
neuroendocrine tumors.
often small, multicentric, and located in the
duodenum, thus diminishing the probability of
surgical cure.
The features predictive of poor prognosis include
pancreatic location of lesions, metastases, ectopic
Cushing syndrome, and high gastrin level.
The development of gastrinomas is preceded by
multifocal hyperplasia of the gastrin-producing cells.
11. Insulinomas
can be multicentric and can metastasize either to
regional lymph nodes or to the liver.
Unlike non-MEN1 insulinomas, usually occur in
patients younger than 40 years and many are
diagnosed in patients younger than 20 years.
Patients often present with hypoglycemic symptoms
after a fast, low blood glucose with symptoms
improving after glucose intake hypoglycemic
symptoms (Whipple’s triad).
12. Glucagonomas
occur rarely (3%) in patients with MEN1
can present silently or with hyperglycemia.
Few patients show the typical skin lesions, known as
necrolytic migratory erythema.
Other presenting symptoms can be anemia,
stomatitis, and weight loss, but these are often absent.
13. A characteristic rash called
necrolytic migratory
erythema seen in the face,
lower abdomen, perineum,
and lower extremities.
14. VIPomas
occur in less than 1% of MEN1 patients.
Presenting symptoms can include watery diarrhea,
hypokalemia, achlorhydria (WDHA syndrome).
Tumors secreting pancreatic polypeptide (PPomas)
may not be associated with clinical manifestations and
may be nonsecretory.
Growth hormone–releasing hormone tumors
(GHRHomas) have been reported in patients with
MEN1 and most commonly arise in the lungs, followed
by the pancreas and small intestine
15. Pituitary tumors
MEN1-associated pituitary tumors most commonly
secrete prolactin (60%), followed by tumors that
secrete growth hormone (25%).
Less than 5% secrete corticotropin and others are
nonfunctional.
Compared with non-MEN1 pituitary tumors, they
tend to be larger (macroadenomas) and more
aggressive, with a higher rate of infiltration of tumor
cells into normal pituitary tissue.
MEN1-associated pituitary tumors are less responsive
to therapy. However, there is no distinct histological
difference between MEN1 and non-MEN1 pituitary
tumors.
16. Other tumors associated with MEN1
Carcinoid tumors can occur in patients with MEN1 and
are located in the bronchi, gastrointestinal tract, pancreas,
and thymus..Carcinoids can actively secrete hormones such
as serotonin, somatostatin, corticotropin, and growth
hormone.
Cutaneous expression of MEN1 is common.
Subcutaneous lipomas are found in one third of MEN1
patients,can also be retroperitoneal, visceral, or pleural.
The presence of facial angiofibromas and collagenomas
may allow presymptomatic diagnosis of MEN1 in relatives
of diagnosed patients.
Adrenal tumors occur and usually are nonfunctional.
They are most often benign 20-40% of patients. Adrenal
cortical carcinomas are rare in MEN1 patients
Thyroid adenomas occur in 5-30% of patients and have
no specific MEN1-reported clinical significance.
Meningiomas and other central nervous system tumors
have been reported.
17. Laboratory Studies
Gastrinomas
Screening should start by age 20 years.
The diagnosis of gastrinoma is confirmed by a
basal serum gastrin level >1000 pg/ml off proton-
pump inhibitors.
A basal gastrin levels >250 pg/ml is suggestive and
can be confirmed with a secretion stimulation test
18. Insulinomas
A supervised 72-hour fast is used most often to confirm
this diagnosis.
Screening should start by age 5 years.
Glucagonomas
Elevated serum glucagon levels and hyperglycemia are
present
Screening should start in children younger than 10 years.
Vasoactive intestinal polypeptidomas (VIPomas)
Elevated serum levels of vasoactive intestinal polypeptide
are present.
Screening should start in children younger than 10 years.
19. Pancreatic polypeptidomas (PPomas)
Pancreatic polypeptide levels are elevated.
Chromogranin A levels can be elevated in any pancreatic
neuroendocrine tumor.
Screening should begin by age 10 years.
Carcinoid tumors
Elevated levels of chromogranin A, calcitonin, corticotropin, or
urinary 5-hydroxyindoleacetic acid (5-HIAA) can occur.
screening is dependent on radiological imaging as no
biochemical abnormality has been consistently observed.
Pituitary tumors
Assess growth hormone levels (insulinlike growth factor-1 [IGF-
1]) and prolactin.
Screening should begin by age 5 years.
Hyperparathyroidism
Calcium is elevated with an inappropriately elevated parathyroid
hormone level.
Screening should start by age 8 years.
20. Imaging Studies
Sagittal (left image) and
coronal (right image), T1-
weighted magnetic
resonance images of the
brain in a patient with
multiple endocrine
neoplasia syndrome type 1
(MEN1). These images
show a pituitary
macroadenoma
21. octreotide scan in a
patient with multiple
endocrine neoplasia
syndrome type 1 (MEN1).
These nuclear images
demonstrate abnormal
activity in the pituitary
macroadenoma (curved
arrow), parathyroid
adenoma (straight arrow),
and gastrinoma
metastases throughout
the abdomen
(arrowheads).
22. (CT) scan of the
pancreas in a patient
with multiple endocrine
neoplasia syndrome
type 1 (MEN1) and a
gastrinoma. This image
shows a pancreatic head
mass (large, white
arrow), as well as a low-
attenuating lesion in the
liver (small, black
arrowhead) that
indicates metastases
23. (CT) scan image with
oral and intravenous
contrast in a patient
with biochemical
evidence of
insulinoma. The 3-cm
contrast-enhancing
neoplasm (arrow) is
seen in the tail of the
pancreas (P) posterior
to the stomach (S)
24. Endoscopic
ultrasonogram in a
patient with an
insulinoma. The
hypoechoic neoplasm
(arrows) is seen in the
body of the pancreas
anterior to the splenic
vein (SV).
25. •99mTc MIBI) in a patient
with multiple endocrine
neoplasia syndrome type 1
(MEN1). These images
demonstrate persistent
abnormal activity of the
inferior right parathyroid
gland that is consistent with
an adenoma.
26. Anteroposterior
radiographic view of the
right hand in a patient
with multiple endocrine
neoplasia syndrome type 1
(MEN1) and primary
hyperparathyroidism. This
image shows subperiosteal
bone resorption along the
radial aspects of the
middle phalanges
(arrows).
28. Approach Considerations
Hyperparathyroidism
Surgery is the definitive treatment. Subtotal (3.5
glands)or total parathyroidectomy with forearm
autotransplantation is performed with an open
bilateral neck exploration. The recommended timing
of surgery in a patient is controversial. Recurrent
hypercalcemia is common. Transcervical thymectomy
may need to be performed at the same time, owing to
the mortality associated with malignant carcinoid
tumors of the thymus.
29. Gastrinoma
Inhibition of acid hypersecretion is achieved with
proton pump inhibitors. Histamine receptor
antagonists may be added.
Nonmetastatic gastrinomas located in the pancreas
are rare but can be surgically excised.
Removal of tumors larger than 2 cm in diameter
reduces the frequency of liver metastasis, which is an
important prognostic factor.Surgical cure of multiple
duodenal gastrinomas is difficult
Other novel approaches, such as chemotherapeutic
agents or hormonal therapy with somatostatin
analogs, can be considered to treat disseminated
gastrinomas.
30. Insulinoma
Surgical removal of the tumor is the treatment of
choice.
Unresectable tumors can be treated with diazoxide or
octreotide. Chemotherapeutic agents or hepatic artery
embolization has been used to treat metastatic disease
Some authors recommend subtotal pancreatectomy
(80% or more of the pancreas) in patients with
multiple tumors or when the tumor is not localized.
31. Glucagonomas
Surgical removal of the tumor is the treatment of choice.
Usually, this involves excision of the tail of the pancreas.
However, often times, metastases have already occurred at the
time of diagnosis. Somatostatin analogs (lanreotide or
octreotide), chemotherapeutic agents, and hepatic artery
embolization have also been used
Vasoactive intestinal polypeptide tumor (VIPoma)
Somatostatin analogs control symptoms in 80% of cases,
although surgical cure should be attempted.
Asymptomatic nonfunctioning pancreatic
neuroendocrine tumors
A consensus regarding surgical indications has not been
established. The goal is to reduce mortality and morbidity
associated with metastatic disease while preserving pancreatic
tissue. Conflicting expert opinion suggests surgical removal of
tumors greater than 1 cm versus 2 cm.
32. Pituitary tumors
Treatment is similar to non-MEN1–associated pituitary
tumors. Prolactinomas are treated with bromocriptine or
cabergoline. Octreotide or lanreotide is used for
somatotrophinomas. Transsphenoidal tumor removal with
radiotherapy can be used if necessary.
Carcinoid tumors
If resectable, surgery is the treatment of choice. For
unresectable tumors, treatment with radiotherapy or
chemotherapeutic agents can be used. Somatostatin
analogs can help with symptoms and may shrink some
tumors.
Cutaneous manifestations of MEN1
Management is conservative for lipomas, facial
angiofibromas, and collagenomas. Local excision can be
performed if desired.
33. SOMATOSTATIN ANALOGS
Used since 1980’s
Hormone blocking agents that are synthetic somatostatin
derivatives (ex: octreotide and lanreotide)
Octreotide acts primarily on somatostatin receptor
subtypes II and V. It inhibits growth hormone secretion
and has a multitude of other endocrine and nonendocrine
effects, including inhibition of glucagon, vasoactive
intestinal peptide, and GI peptides.
Side effects: development of gallstones secondary to
inhibition of cholecystokinin release, pain at site, hypo or
hyperglycemia, rash, alopecia, fluid retention
34.
35. Type 2 multiple endocrine
neoplasia (MEN 2)
Sipple first described an association between thyroid
cancer and pheochromocytoma in 1961.
The thyroid cancer found with pheochromocytoma
was discovered in 1965 to be a medullary carcinoma
characterized by stromal amyloid.
In 1968, this familial constellation of pathology in
conjunction with parathyroid hyperplasia was
recognized as (MEN 2).
the distinction between MEN 2A and MEN 2B was not
made until 1975
36. Phenotype - MEN 2A patients do not have the
phenotypic abnormalities of mucosal neuromas and
marfanoid habitus found in MEN 2B patients
Medullary thyroid carcinoma - MEN 2A patients have
a less virulent than do MEN 2B patients
Parathyroid hyperplasia - MEN 2A patients may have,
which is exceedingly rare in MEN 2B patients
A third subtype of MEN 2 is familial medullary thyroid
carcinoma only (FMTC only).
37. Etiology
MEN 2 is a rare familial cancer syndrome caused by
mutations in the RET proto-oncogene localized to
10q11.2 .
Inherited as an autosomal dominant disorder,
38. Pathophysiology and presentations
Medullary thyroid carcinoma
Virtually in all patients with MEN 2A.
the first expressed abnormality and usually occurs in the second or third
decade of life.
typically bilateral and multicentric, in contrast to sporadic medullary
thyroid carcinoma, which is unilateral.
Pheochromocytoma
Pheochromocytomas are present in 50% of MEN 2A patients.
bilateral in 60-80% of patients, compared with 10% of patients with sporadic
pheochromocytomas.
diagnosed at the same time as medullary thyroid carcinoma or several years
later (with both occurring primarily in the second or third decade).
nearly all benign.
Even so, these lesions can cause a life-threatening hypertensive episode or
arrhythmia.
Parathyroid hyperplasia
in nearly half of patients with MEN 2A but are less common than
pheochromocytomas.
can be clinically silent.
.
39. Hypertension
If pheochromocytomas develop, an increase in blood
pressure and heart rate may be the only signs. These
increases can be chronic or episodic. Some patients have
episodes of sweating and headaches.
Diarrhea
If a patient has medullary thyroid carcinoma
This may be related to elevated prostaglandin or
calcitonin levels.
Pruritic skin lesions
Cutaneous lichen amyloidosis in MEN 2A patients
manifests as multiple pruritic, hyperpigmented,
lichenoid papules in the scapular area of the back..
40.
41. The marfanoid habitus of high-arched palate,
pectus excavatum, bilateral pes cavus, and scoliosis are
observed in MEN 2B patients.
Neuromas on the eyelids, conjunctiva, nasal and
laryngeal mucosa, tongue, and lips are frequent
findings.
Patients also have prominent, hypertrophied lips
leading to a characteristic facies. Localized pruritus
appears over the upper back in MEN 2B patients.
42.
43. Approach Considerations
Perform genetic screening for RET mutations in all index
patients. If a mutation is identified, also screen family
members who are at risk.
baseline calcitonin levels and of serum calcium and
parathyroid hormone (PTH) levels, along with urine
collection for catecholamines and metanephrine
concentrations.
Imaging Studies
Perform computed tomography (CT) scanning or magnetic
resonance imaging (MRI) of the adrenals.
A metaiodobenzylguanidine (MIBG) scan is useful for
localizing pheochromocytomas
44. Fine-needle aspiration
Avoid the removal of cells from thyroid masses for
cytology in patients with type 2 multiple endocrine
neoplasia (MEN 2) who have had their diagnosis
previously confirmed by either genetic analysis or
elevated calcitonin levels as biopsy increases the
possibility of tumor spread
45. Management of comlications
Pheochromocytoma
Preoperatively, prepare patients by treating them with an
alpha-blocker or a tyrosine hydroxylase inhibitor, such as
metyrosine, for 1-2 weeks, after which administration of a
beta-blocker can be considered
Hypercalcemia
Patients presenting with severe hypercalcemia should first be
hydrated, after which they should be treated with
furosemide. If they remain severely hypercalcemic,
consider treatment with calcitonin, glucocorticoids, or
bisphosphonates (such as pamidronate).
46. Treatment strategy
Surgical treatment is the standard with replacement
therapy after the surgey
Patients require hormone replacement following total
thyroidectomy and bilateral adrenalectomy or when
they have postoperative hypoparathyroidism. In
addition, patients who develop postoperative
hypoparathyroidism need supplemental calcium
and/or vitamin D.
The corticosteroid cortisone and the mineralocorticoid
fludrocortisone acetate can be used in combination in
patients suffering from adrenocortical insufficiency.