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Unusually young age distribution of primary hepatic leiomyosarcoma: case series and review of the adult literature.

Shamseddine A, Faraj W, Mukherji D, El Majzoub N, Khalife M, Soubra A, Shamseddine A - World J Surg Oncol (2010)

Bottom Line: One patient is currently 12 months post-resection with no evidence of recurrence.Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.These cases highlight the diagnostic and therapeutic challenges of this rare tumour.

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Affiliation: Department of Surgery, HPB and Liver Transplantation Unit, American University of Beirut Medical Center, Beirut, Lebanon.

ABSTRACT

Background: Primary hepatic leiomyosarcoma is a rare disease diagnosed in older aged adults with a median age of 58 and occasionally in children with a history of immunosuppression.

Methods: From 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; median age 30 (range 20-39) years.

Results: One patient is currently 12 months post-resection with no evidence of recurrence. Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.

Conclusion: We have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour.

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CT scan of patient number 3 with primary hepatic sarcoma.
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Figure 1: CT scan of patient number 3 with primary hepatic sarcoma.

Mentions: The third patient was a 30-year old male who presented with abdominal pain associated with anorexia, weight loss, nausea and vomiting. A CT scan showed a large multi-lobulated lesion measuring 18 × 17.8 × 11.8 cm occupying almost the entire right hepatic lobe (figure 1). It appeared heterogeneous with cystic and soft tissue components. The initial diagnosis was of hydatid disease of the liver. A right hepatectomy was performed and histopathological examination defined the tumor as a stage IV LMS with metastasis to the abdominal wall. Immunohistochemistry showed diffusely positive staining for MSA, weakly positive for vimentin, and strongly and diffusely positive for SMA. Adjuvant chemotherapy was recommended and the patient continued his treatment at another institution. He received six cycles of chemotherapy and 30 sessions of radiotherapy. The patient is currently 12 months post-resection with no evidence of recurrence.


Unusually young age distribution of primary hepatic leiomyosarcoma: case series and review of the adult literature.

Shamseddine A, Faraj W, Mukherji D, El Majzoub N, Khalife M, Soubra A, Shamseddine A - World J Surg Oncol (2010)

CT scan of patient number 3 with primary hepatic sarcoma.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2902476&req=5

Figure 1: CT scan of patient number 3 with primary hepatic sarcoma.
Mentions: The third patient was a 30-year old male who presented with abdominal pain associated with anorexia, weight loss, nausea and vomiting. A CT scan showed a large multi-lobulated lesion measuring 18 × 17.8 × 11.8 cm occupying almost the entire right hepatic lobe (figure 1). It appeared heterogeneous with cystic and soft tissue components. The initial diagnosis was of hydatid disease of the liver. A right hepatectomy was performed and histopathological examination defined the tumor as a stage IV LMS with metastasis to the abdominal wall. Immunohistochemistry showed diffusely positive staining for MSA, weakly positive for vimentin, and strongly and diffusely positive for SMA. Adjuvant chemotherapy was recommended and the patient continued his treatment at another institution. He received six cycles of chemotherapy and 30 sessions of radiotherapy. The patient is currently 12 months post-resection with no evidence of recurrence.

Bottom Line: One patient is currently 12 months post-resection with no evidence of recurrence.Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.These cases highlight the diagnostic and therapeutic challenges of this rare tumour.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgery, HPB and Liver Transplantation Unit, American University of Beirut Medical Center, Beirut, Lebanon.

ABSTRACT

Background: Primary hepatic leiomyosarcoma is a rare disease diagnosed in older aged adults with a median age of 58 and occasionally in children with a history of immunosuppression.

Methods: From 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; median age 30 (range 20-39) years.

Results: One patient is currently 12 months post-resection with no evidence of recurrence. Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.

Conclusion: We have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour.

Show MeSH
Related in: MedlinePlus