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Retroperiton masses


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Retroperiton masses

  1. 1. Pathology of Retroperitoneal Mass
  2. 2.   The retroperitoneal space is a potential space extending posterior to the abdominopelvic cavity. The tissue within this mixed potential and actual space is composed of:  Lymphatics and loose connective tissue.  Organs that are partially or completely retroperitoneal include: • • • • Pancreas, kidney, Adrenal glands, Ureters.  Vascular structures: • Aorta and inferior vena cava.  Significant lumbar nerves traverse the space.
  3. 3. Differential diagnosis of retroperitoneal soft tissue mass The term retroperitoneal tumour is at times confined to the lesions arising from tissues (muscles, fat, fibrous tissue, lymph nodes, nerves and developmental remnants) of this compartment but excluding origin from the retroperitoneal organs. Malignant lesions: • • • • Lymphoma (most common) Sarcoma (2nd most common) GIST Germ cell tumor Desmoid tumors. Benign lesions: • • • • Lipoma Peripheral nerve sheath tumor Teratoma Paraganglioma
  4. 4. Diagnostic Work-Up  Computed tomography (CT):      Large lesions with fatty components causing mass effect frequently represent liposarcomas. Teratomas often have fat, fluid, and calcified components. Paraspinal locations point to nerve sheath tumors or neurogenic derivation . Magnetic resonance imaging (MRI) may demonstrate additional imaging details to further classify the lesion. CT-guided needle biopsy may be performed as the initial means of obtaining tissue for histologic diagnosis
  5. 5. Retroperitoneal Sarcoma  Second most common malignant RPT.  Arise from: nonepithelial, extraskeletal tissues: (fat, muscle, nerve and nerve sheath, blood vessels, other connective tissues).  Incidence: 9,220 cases per year in the United States.  Common types:    Leiomyosarcoma, Liposarcoma, Fibrosarcoma.
  6. 6. ‫‪Retroperitoneal sarcoma‬‬ ‫ــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــ‬
  7. 7. Retroperitoneal sarcoma ‫ــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــ‬  Primary treatment of retroperitoneal soft tissue sarcoma (STS) is to attempt a gross total resection.  Criteria for unresectable tumors commonly include major vessel invasion and spinal cord or vertebral body involvement.  Resectability has been reported in recent series to range from 65% to 85%.
  8. 8. Retroperitoneal sarcoma ‫ــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــ‬  Macroscopically positive margin is one the most important prognostic features in determining local control and survival.  Even after a complete excision of RPS, local recurrence rates (33% to 77%) and overall survival rates (35% to 63% 5-ys OS) are poor.  retrospective data demonstrate a decrease in local failure with adjuvant radiation therapy.  The use of adjuvant and neoadjuvant chemotherapy is not standard.
  9. 9. Retroperitoneal sarcoma ‫ــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــ‬ Patients with unresectable disease: • May benefit from a course of preoperative radiotherapy or chemotherapy in attempt to shrink the tumor. • Palliative debulking surgery may be offered. Patients with metastatic disease:  Solitary or limited metastases: should be managed similarly to those with localized disease with consideration for preoperative radiation therapy and/or chemotherapy, metastasectomy, radiofrequency ablation. Chemotherapy may be considered (most commonly, Doxorubicin-based regimens).  Disseminated metastatic disease: treated with palliative intent (chemotherapy, radiation therapy, palliative surgery or best supportive care.)
  10. 10. GIST gastrointestinal stromal tumour  These tumors that derive from the precursors of the interstitial cells of Cajal may occur anywhere in the GI tract, but most often from the stomach and small intestine.  incidence is estimated to be approximately 1.5/100,000 per year.  Approximately 95% of GISTs are CD117 (c-KIT) positive.
  11. 11. GIST ‫ـــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــــ‬  Surgery: • Remains the only potentially curative treatment for patients with localized GIST. • Optimal surgery requests complete excision of the tumor without rupture in order to avoid seeding of tumor cells. • No role in advanced or metastatic disease. Imatinib (targeted therapy): • Tyrosine kinase inhibitor of c-KIT. • Adjuvant: in tumors > 3 cm, for 1 year. • Upfront therapy: in advanced and metastatic cases.
  12. 12. Desmoid tumors        (aggressive fibromatosis) Rare, benign neoplasms. Arise from muscle fascia, aponeuroses, tendons, and scar tissue. More commonly in females . Associated with familial adenosis polyposis . Tend to be locally aggressive and have a propensity to recur locally after resection. Treatment is complete resection with a 2-3 cm margin. Postoperative radiation therapy is used in cases with an unresectable primary, after multiple local recurrences.
  13. 13. Schwannomas  Most common benign tumors in the retroperitoneum.  Typically form large, well-circumscribed masses (may display cystic degeneration, calcification, hemorrhage).  MRI is the recommended imaging study.  Not suggest CT-guided biopsy or fine-needle aspiration for the diagnosis because of the risk of hemorrhage as it is usually a highly vascular tumor.  Complete surgical resection with negative margins is the treatment of choice.