DEMOPLASTIC TUMOR OF SMALL ROUND CELLS - A CASE REPORT IN BRAZILIAN NORTHWEST

Case Report: Patient C.R.S.A, brown, 40 years old, obese, born in Manaus - AM, was treated with pain in the left flank of the colic type with gradual evolution for 3 years. It refers to the presence of palpable mass in the same location. Former chronic alcoholic for 20 years, denies smoking. Reported use of subcutaneous Lipostabil for two months 5 years ago. Total abdominal Doppler ultrasound was performed and showed a nodular hypoechoic image in the periumbilical area, with irregular borders and peripheral vascularization. Abdominal and pelvic tomography revealed peritoneal expansive formation in contact with the ileum loop and nodules suggestive of pelvic peritoneal implants and affected lymph nodes. He underwent laparotomy for resection of the mass firmly adhered to an epiplon segment, measuring 1,718 g and measuring 20×19×13 cm. The histopathological report showed poorly differentiated neoplasia with areas of sarcomatous aspect along with areas of necrosis. In immunohistochemistry, malignant neoplasia of small solid blocks, small, monomorphic round oval cell nests and cords was noted, favoring the diagnosis of Small Round Cell Desmoplastic Tumor (TDPCR). Oncologic PET-CT 60 days after surgery showed expansive nodules, located in vesico-rectal peritoneal reflection, mesenteric lymph nodes and soft tissue lesions. Resection of metastatic foci evidenced by PET-CT was chosen. Asymptomatic patient, remains under clinical observation and outpatient follow-up every 6 months. Discussion: TDPCR mainly affects the intra-abdominal cavity and is more common in male children and young adults. The main form of presentation is multiple nodules adhered to the abdominal surface. It is a rare neoplasm, with just over 100 cases described in the literature, the main foci of peritoneum, liver and lymphoid tissue metastasis. The diagnosis is made by immunohistochemistry and the prognosis is poor due to poor response to chemotherapy and radiotherapy mainly in late diagnoses when the tumor is unresectable, unlike the case in which surgical resection was possible. Final comments: It is noted the rarity of the case and the importance of registering and discussing similar clinics in order to standardize drug and surgical treatment protocols, in order to provide targeted therapy and increased survival of patients with quality of life.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
23 October 2019