Electronic Clinical Challenges and Images in GI

An Extremely Unusual and Large Cause of Anemia

Javier G. Castillo,* Dana Telem, and Tomas M. Heimann
*Departments of Thoracic Surgery and General Surgery, James J. Peters VA Medical Center, Bronx; The Mount Sinai School of Medicine, New York, New York

Question: A 63-year-old man with a long-standing medical history of anemia presented to the gastroenterology outpatient clinic
with diffuse abdominal pain, progressive dragging sensation in the left upper quadrant, and marked dizziness. Other prominent
clinical symptoms included a weight loss of 15 lbs over 2 months, anorexia, and intermittent constipation. Clinical examination
revealed tenderness in the left upper quadrant and massive splenomegaly. On further palpation, the spleen extended to the
umbilicus, and had a smooth surface and a very firm consistency. The patient was hemodynamically stable with an arterial blood
pressure of 111/56 mmHg and a heart rate of 90 beats/min. A complete blood cell count showed anemia (hemoglobin, 5.7 g/dL)
and a normal platelet count. No abnormalities were found on the hemogram on a peripheral blood smear. After initial resuscitation,
malignancy screenings, abdominal ultrasonography and computed tomography were performed. Ultrasonographic examination
showed a large heterogeneous mass pattern measuring 30 cm (Figure A). The liver was mildly enlarged with no dilated intrahepatic
ducts. Subsequent abdominal computed tomography confirmed the ultrasonographic findings (Figure B) and further revealed
coarse calcifications, hypodense areas, and enhancing septations (Figure C).
What is the diagnosis?
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Clinical Challenges and Images in GI.
Conicts of interest: The authors disclose no conicts.
2012 by the AGA Institute
0016-5085/$36.00
doi:10.1053/j.gastro.2011.03.067

GASTROENTEROLOGY 2012;142:e23 e24

Electronic Clinical Challenges and Images in GI, continued

Answer to the
Clinical Challenges and Images
in GI Question:
Image 11: Angiosarcoma of
the Spleen
Splenic artery embolization was performed before surgical en block
splenectomy (Figure D,
E). Surgical exploration
demonstrated a normal
upper pole of the spleen
encased in peritoneum.
The specimen (Figure F)
measured 30 24 8
cm, weighed 3690 g,
and comprised a nodular and spongy tumor
with hemorrhagic characteristics. Histopathology showed an angiomatous neoplasm with
irregular vascular channels, atypical endothelial and spindle cells, as
well as extensive necrosis with GamnaGandy
bodies (Figure G). Immunohistochemical
studies confirmed the
suspicion of angiosarcoma of the spleen. The mitotic count was 12 per 10 high-power fields.
Primary angiosarcoma of the spleen is an extremely rare mesenchymal neoplasm with an incidence of 0.14 0.25 cases per million
population.1 The mean age of patients at diagnosis is 59 years. There is not a clear gender predilection, although a slight
predominance in men has been reported. Although the natural history and pathology of this tumor remains unknown, ionizing
radiation, arsenic poisoning, vinyl chloride, chemotherapy for lymphoma, previously existing benign lesions such as hemangioma
or hemangioendothelioma, or even foreign body tumorigenesis have been hypothesized as possible causes. In our particular case, no
such preexisting tumors or risk factors were present.
Clinical manifestations are very variable and range from nonspecific symptoms to life-threatening scenarios such as splenic
rupture and consequent lethal hemoperitoneum, observed in 13%32% of the cases.2 Nonspecific misleading symptoms including
left upper quadrant abdominal discomfort, fatigue, dizziness, weight loss, and anemia, as well as thrombocytopenia are present in
about 65% of patients. Imaging modalities are invaluable to obtain a differential diagnosis and are currently performed to assess
tumor extension. Biopsy is contraindicated in splenic angiosarcoma because of the high risk of rupture.
References
1. Falk S, Krishnan J, Meis JM. Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases. Am J Surg Pathol
1993;17:959 970.
2. Neuhauser TS, Derringer GA, Thompson LD, et al. Splenic angiosarcoma: a clinicopathologic and immunophenotypic study
of 28 cases. Mod Pathol 2000;13:978 987.
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