? Appendiceal malignancies are extremely rare.
? Diagnosed in 0.9-1.4% of appendectomy specimens.
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? Carcinoid is the most common appendiceal malignancy
? It represents more than 50% of the primary lesions of the appendix.
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WHO classification of Appendiceal tumors
Carcinoid
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? Most common site: Jejunum>Appendix > rectum? Mean age at presentation is 32-43 years (range-6 to 80 years)
? More frequently in females than in males
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? Carcinoid syndrome is rarely associated with appendiceal carcinoid
? Symptoms attributable directly to the carcinoid are rare
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? Tumor can occasionally obstruct the appendiceal lumen and result in acute appendicitisMacroscopy
? Firm, greyish-white (yel ow after fixation), fairly wel circumscribed, but not
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encapsulated? Measure usual y less than 1 cm in diameter
? Tumours > 2 cm are rare
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? Most are located at the tip of the appendix
? Goblet-cel carcinoids and mixed endocrine-exocrine carcinomas of the appendix
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may be found in any portion of the appendix.Carcinoid
? Tumours with endocrine differentiation arising in the appendix
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? Majority of carcinoids are located in the tip of the appendix.
? Malignant potential is related to size
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? Treatment :? carcinoid tumor is localized to the appendix: simple appendectomy
? tumors <1 cm with extension into the mesoappendix and tumors >1.5 cm: right
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hemicolectomy
Carcinoid tumour of appendix with typical yel ow colouration
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Adenocarcinoma
? Primary adenocarcinoma: rare neoplasm
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? Three major histologic subtypes:? Mucinous adenocarcinoma
? Colonic adenocarcinoma
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? Adenocarcinoid
TNM Staging
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? Most common mode of presentation is of acute appendicitis.
? Ascites
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? Palpable mass? Incidental intraoperative finding
Treatment:
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? Right hemicolectomy
Appendiceal adenocarcinomas have a propensity for early perforation
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Overall 5-year survival is 55%Patients have significant risk for both synchronous and metachronous neoplasms
Mucocele
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Progressive enlargement of the appendix from the intraluminal accumulationof a mucoid substance.
Mucocele are of four histologic types
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? Retention cysts
? Mucosal hyperplasia
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? Cystadenomas? Cystadenocarcinomas
A mucocele of benign etiology is adequately treated by simple appendectomy
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Pseudomyxoma Peritonei
? Diffuse collections of gelatinous fluid are associated with mucinous implants on peritoneal
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surfaces and omentum.? Two to three times more common in females than males.
? Recent immunocytologic and molecular studies suggest that the appendix is the site of
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origin for most cases of pseudomyxoma.
? Pseudomyxoma is invariably caused by neoplastic mucous-secreting cells within the
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peritoneum.? Clinical presentation: abdominal pain, distension, mass, "jelly-belly"
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? Tumor Markers: CA-19.9, CEA, CA-125? Imaging:
? CT scanning is the preferred imaging modality
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? Role of colonoscopy in the diagnosis of PMP is minimal
Intra operative photograph of patient PMP
Complete Cytoreductive Surgery is the mainstay of treatment
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? All gross disease should be removed
? Appendectomy is routinely performed
? Hysterectomy with bilateral salpingo-oophorectomy is performed in women.
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At surgery a variable volume of mucinous ascites is found together with tumordeposits
Pseudomyxoma is a disease that progresses slowly and in which recurrences may
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take years to develop or become symptomatic.
Hyperthermic intraperitoneal chemotherapy (HIPEC)
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? Highly concentrated? Heated chemotherapy treatment
? Delivered directly to the abdomen during surgery
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? Intraoperative HIPEC was initiated at the Washington Hospital Center in
1992
Lymphoma
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? Extremely uncommon
? Gastrointestinal tract is the most frequently involved extranodal site for
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non-Hodgkin's lymphoma? Frequency of primary lymphoma of the appendix ranges from 1 to 3% of
gastrointestinal lymphomas
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? Usually presents as acute appendicitis
Lymphoma
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? CT scan showing appendiceal diameter greater than or equal to 2.5 cm orsurrounding soft-tissue thickening should prompt suspicion of an
appendiceal lymphoma
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? management of appendiceal lymphoma confined to the appendix is
appendectomy.
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? Right hemicolectomy is indicated if there is extension of tumor beyond theappendix onto the cecum or mesentery.