Rare Coincidence of Familial Adenomatous Polyposis and a Retroperitoneal Fibromyxoid Sarcoma: Report of a Case

Abstract

To our knowledge, this is the first reported case of familial adenomatous polyposis with metachronous retroperitoneal fibromyxoid sarcoma. Proctocolectomy or total colectomy and complete tumor resection is the treatment of choice in this case. In addition to more common semimalignant retroperitoneal desmoid tumors in familial adenomatous polyposis patients, a malignant soft-tissue tumor also has to be considered for differential diagnosis.

Multiple Neoplasia in a 15-year-old Girl With Familial Adenomatous Polyposis

“…2 A case of low-grade fibromyxoid sarcoma in retroperitoneal is also reported in a 24-year-old man. 8 TC has an estimated incidence of 1% to 2% in FAP patients. 9 All the cancers seen in this patient have been reported in FAP patients but the young age of appearance of these neoplasias in this girl is uncommon.…”

Different Clinical Manifestations Between Primary Gastrointestinal Malignancies and Benign Tumors in Children

“…To date, there have been only a few cases of FAP-associated sarcoma, and the etiology is still unknown. Genetic factors may be one of the contributory factors (29). Lastly, intestinal fibrosarcoma is also extremely rare, with only a few case reports published to date (30)(31)(32).…”

Retroperitoneal low‐grade fibromyxoid sarcoma

“…LGFMS comprises deceptively bland‐appearing spindle‐shaped cells in a combination of collagenous and myxoid stroma 1–5 . The disease causes delayed local recurrence and metastasis; however, in a small number of cases, surgical resection provides a long‐term prognosis.…”

“…LGFMS comprises deceptively bland‐appearing spindle‐shaped cells in a combination of collagenous and myxoid stroma. 1 , 2 , 3 , 4 , 5 The disease causes delayed local recurrence and metastasis; however, in a small number of cases, surgical resection provides a long‐term prognosis. In this case report, we described a rare case of LGFMS arising in the retroperitoneum as well as present a literature review.…”