Primary hepatic leiomyosarcoma in a patient with autosomal dominant polycystic kidney disease

CEN Case Reports
Takashi IidaFumitaka Nakajima

Abstract

Primary hepatic leiomyosarcoma is an extremely rare tumor. The diagnosis is difficult, and its etiologic factors have not been clarified. A 63-year-old woman with numerous cysts in her kidneys and liver was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). Several members of her family also had ADPKD. She underwent treatment with tolvaptan to inhibit cyst growth and slow the decline in kidney function. Eight months after the start of the therapy, she was hospitalized with fatigue and fever of unknown origin. Diagnostic imaging showed a very large hepatic tumor, and histologic examination of a fine-needle biopsy specimen revealed the tumor to be malignant. Differentiation between carcinoma and sarcoma was difficult based on the histological findings. The tumor was thought to be excisable; therefore, hepatic resection was attempted. At the time of surgery, as the tumor had grown larger than when imaged, complete resection was impossible. However, a part of the tumor was resected. Histopathological and immunohistological examinations of the surgical specimen confirmed a primary hepatic leiomyosarcoma. Whether the tumor was associated with the presence of ADPKD remains unclear, however, this is the first report o...Continue Reading

References

Oct 1, 1987·Archives of Surgery·H S MakiM E Kuehner
Dec 1, 1994·Diagnostic Cytopathology·M B SmithK R Geisinger
Jan 1, 1995·Cancer·M T Carriaga, D E Henson
Mar 1, 1996·Abdominal Imaging·F FerrozziG Garlaschi
Jun 14, 2002·Surgery Today·Hideto FujitaKishichiro Watanabe
Jul 26, 2002·Gastroenterología y hepatología·P Linares TorresJ L Olcoz Goñi
Jul 9, 2004·Nihon Ronen Igakkai zasshi. Japanese journal of geriatrics·Kyoko MarutaHidetoshi Fukunaga
Feb 23, 2007·Cancer·Juürgen WeitzRonald P Dematteo
Nov 3, 2011·World Journal of Gastrointestinal Oncology·Nairuthya ShivathirthanKeiichi Kubota
Nov 6, 2012·The New England Journal of Medicine·Vicente E TorresUNKNOWN TEMPO 3:4 Trial Investigators
Aug 8, 2013·Journal of Gastrointestinal Cancer·Humberto MettaMarcela Carballido
Nov 12, 2013·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Randy L Luciano, Neera K Dahl

❮ Previous
Next ❯

Citations

Jan 22, 2019·Acta Medica (Hradec Králové)·Themistoklis FeretisDimitrios Dimitroulis
Mar 18, 2020·Annals of Hepato-biliary-pancreatic Surgery·Francesco EspositoDaniel Azoulay

❮ Previous
Next ❯

Related Concepts

Related Feeds

Carcinosarcoma

Carcinosarcoma is a malignant neoplasm that contains elements of carcinoma and sarcoma so extensively intermixed as to indicate neoplasia of epithelial and mesenchymal tissue. Discover the latest research on carcinosarcoma here.

Related Papers

The New England Journal of Medicine
Vicente E TorresTEMPO 3:4 Trial Investigators
The New England Journal of Medicine
Vicente E TorresFrank S Czerwiec
© 2022 Meta ULC. All rights reserved