Dados do Trabalho

Título

Dedifferentiated liposarcoma of the gallbladder: First reported case

Apresentação do Caso

A 71-year-old woman was referred to the Surgical Oncology Division due to a solid mass at the right side of the abdomen and fever, with no signs of jaundice. Abdominal ultrasonography and computed tomography (CT) evidenced an extensive gallbladder lobular formation adhered to the inferior border of the right hepatic lobe and cholelithiasis. The CT report suggested gallbladder liposarcoma. A cholecystectomy associated with resection of segments IV-B and V of the liver were performed. Intraoperative frozen sections were compatible with gallbladder sarcoma. Anatomopathological examination and immunohistochemistry confirmed dedifferentiated liposarcoma with foci of heterologous leiomyosarcomatous differentiation and undifferentiated fusocellular areas of high histological grade.

Discussão

Liposarcoma (LPS) of the gallbladder is one of the least common sarcomas, with only five cases reported in the literature according to our knowledge. The mean age at diagnosis of LPS among the five cases reported in the literature and ours is 67.17 ± 10.11 years, and there is a prevalence of 83.33% of female patients.
Regarding the localized disease, surgical resection with clear margins remains to be the mainstay of treatment for LPS. The use of systemic therapy does not improve the course of the disease, except in cases of myxoid liposarcoma, which is more chemosensitive and radiosensitive than the others subtypes. Similarly to the pleomorphic liposarcoma, the dedifferentiated liposarcoma is a high grade and aggressive tumor, typically chemoinsensitive and radioinsensitive, in which cases the benefit of systemic therapy has been reported to be minimal.
The prognosis of patients with gallbladder liposarcoma is poor, because these are tumors with an aggressive behavior and, generally, present high rates of recurrence and metastasis. Taking this scenario into consideration, it is expected that, in the future, the patient of our case may require further surgical procedures in order to resect possible sites of metastasis, such as happened in the cases described by Hamada et al. and Bader et al.

Comentários finais

To sum up, among the five cases of gallbladder liposarcoma ever reported in the literature to date, only two histological types were identified (1 pleomorphic liposarcoma and 4 myxoid liposarcoma). Our case stands as the first case of subtype dedifferentiated liposarcoma of the gallbladder to be reported.

Palavras-Chave

Gallbladder Disease; Gallbladder Neoplasms; Immunohistochemistry; Liposarcoma; Dedifferentiated Liposarcoma.