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Primary angiosarcoma of the spleen.

Jun-Te Hsu¹, Han-Ming Chen, Chin-Yew Lin

¹Department of General Surgery, Chang Gung Memorial Hospital, Taoyuan, Taiwan.

Journal of Surgical Oncology

|November 22, 2005

Summary

Primary splenic angiosarcoma is a rare cancer. This study reports six cases, finding splenomegaly common and highlighting potential long-term survival after splenectomy alone.

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Area of Science:

Oncology
Pathology
Surgical Oncology

Background:

Primary splenic angiosarcoma is an exceptionally rare and aggressive vascular neoplasm.
It is characterized by a high propensity for metastasis and a poor prognosis.
Limited case reports necessitate further clinical data for understanding and treatment.

Purpose of the Study:

To report six additional cases of primary splenic angiosarcoma.
To analyze the clinical presentation, treatment, and outcomes of these patients.
To contribute to the limited medical literature on this rare malignancy.

Main Methods:

Retrospective review of primary splenic angiosarcoma cases treated at Chang Gung Memorial Hospital (April 1991 - July 2004).
Analysis of patient demographics, clinical symptoms, hematological findings, and imaging data.

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Evaluation of treatment modalities (splenectomy, hepatectomy, chemotherapy) and survival rates.

Main Results:

Six cases (3 male, 3 female; age 7-69) were identified, with common symptoms including abdominal masses/pain, fatigue, and weight loss.
Splenomegaly was universal; anemia and thrombocytopenia were frequent hematological findings.
At diagnosis or post-therapy, metastasis to liver, bone, and bone marrow occurred. Survival rates at 1, 3, and 5 years were 60%, 40%, and 40%, respectively, with a median survival of 36 months.

Conclusions:

Clinical presentation aligns with prior reports, with a notable increase in splenomegaly incidence.
Splenectomy alone may lead to long-term disease-free survival, even in adult cases.
This study adds valuable data on the management and prognosis of primary splenic angiosarcoma.