JBR–BTR, 2011, 94: 287-289.DUODENAL METASTASIS OF ALVEOLAR SOFT PART SARCOMAI. Willekens1,2, C. Paradisi3, L. Sarria3, A. Puertas4, J. Pac4, E. Mayayo4
Aveolar soft part sarcoma is a rare tumor responsible for about 1% of all soft tissue sarcomas, affecting mostly adolescents and young adults. ASPS has curious patterns of metastatic spread, with seldom lymph node involvement. Lung, bone and brain are the most common metastatic places. Small bowel metastasis are infrequent,having found reported only one case of duodenal metastasis with polypous appearance. We describe a case of duodenal metastasis presenting as abdominal mass five years after initial diagnosis of alveolar soft part sarcoma
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Discussion
Alveolar soft part sarcoma (ASPS)was first described by Christophersonet al. in 1952 (1). It is a rare type ofsoft tissue malignant tumor whichaccounts for about 1% of all soft tis-sue sarcomas (2). The disease affectsmostly adolescents and youngadults, with a lower age at diagnosiscompared to other forms of soft tis-sue sarcoma. The peak incidence isdescribed between 15 and 35 yearsold (3). There is a female predilec-tion, especially during the first twodecades of life (4). In adults it mostcommonly involves the muscle and15 years before and multiple lungand brain metastases. She wasfound to have caecal metastases,revealed by anaemia, and was treat-ed by laparoscopic right colecto-my (12). In 2009, Banihani et al. pub-lished a case about a 38-year-oldman with a huge abdominal massinfiltrating the omentum.Pathological diagnosis was ASPS.He had metastases in both lungs andthe right atrium. Afterwards multiplesessile polyps also appeared instomach and duodenum with diag-nostic biopsy of ASPS. Finally thepatient developed brain metastasesand died (13).Primary gastrointestinal ASPS isextremely rare. Only one case hasbeen reported in 2000 by Yaziji et al.,a primary ASPS of the stomach in a54-year-old Italian woman withoutevidence of primary neoplasm else-where ten years following the initialdiagnosis (14).In metastatic tumors, small bowelinvolvement is uncommon and hasbeen described in only 2% of autop-sy cases. Secondary tumors involv-ing the duodenum can arise fromperitoneal dissemination, directspread from an intra-abdominalmalignancy, hematogenous andlymphatic spread (15). Commonmetastatic malignancies known toinvolve the small intestines aremelanomas, lung cancer (16), cervixcarcinoma, renal cell carcinoma, thyroid carcinoma, hepatocellularcarcinoma and Merkel cell carcino-ma (17). The incidence increaseswith age and males are more com-monly affected. Metastatic lesions ofthe duodenum mostly locate in theperiampullar region, followed by theduodenal bulb. Patients present withabdominal pain, nausea, vomitingand gastrointestinal bleeding (18).The microscopic picture of ASPSis uniform and characterized by apseudoalveolar pattern with nests oftumor cells separated by sinusoidalvascular channels. The cells havevesicular nucleoli and eosinophiliccytoplasm (3).Magnetic resonance imaging isthe best technique for characteriza-tion of ASPS. Common MRI findingsare high-signal-intensity on T1 andT2-weighted images and multipleintra- and extra-tumoral signal voids.The high-signal-intensity areas ofthe tumors on T1-weighted sequencecan be attributed to slow flowingblood in or around the tumor (19). Presence of metastases at thetime of diagnosis carries a poorerprognosis (median survival time of3 years), while early metastases dodeep soft tissue of extremities, trunk,head, neck and retroperitoneum.Head and neck locations are morecommonly affected in children andadolescents (5, 6). ASPS has unusual patterns ofmetastatic spread (7). Metastasisoccurs in about 68% of cases and isprimarily haematogenous, withrarely lymph node association (8).The most common metastatic placesare lung, bone and brain (9).The gastrointestinal tract isuncommonly affected by ASPSmetastases. Only four cases of ASPSintestinal metastases has beenreported in literature so far, the firstby Sueyoshi in 1996, affectingjejunum with gastrointestinal bleed-ing associated (10). In 2001, Sabel etal. described a case in small bowel,causing polyposis and intussusep-tion in a 42-year-old male with previ-ous history of ASPS metastatic tolung and brain (11). Zilber et al. in2003 found the first case of colicmetastases in a 43-year-old womanwith a leg primary... "
Brittany is one of the patients with the rare diagnosis of a duodenal met which was found at the same time as her pancreatic and small bowel intussusception mets were diagnosed, but thankfully they all seemed to have completely disappeared since she began her Cediranib treatment.