Friday, June 19, 2015

Interesting Case June 2015 #2

Case History: 

A 62 year old woman with a history of renal cell carcinoma removed years ago presented with a 2.4 cm liver mass found in CT during follow-up. A partial hepatectomy was performed.

Laboratory values: 

ALT, AST, alkaline phosphatase and bilirubin were normal. There is no prior history of liver disease.

Gross examination of the liver resection: 

Cut section reveals a 2.4 cm circumscribed mass with a gray-brown focally spongy appearance.


Figure 1

Figure 2

Figure 3, CD31 immunohistochemistry

Final diagnosis: 

Hemangioma, anastomosing type.

Discussion:

Hepatic hemangioma is a common benign vascular neoplasm in both infants and adults that shares the same growth pattern and prognosis as its cutaneous counterpart. Based on its distribution, hemangioma can be classified as focal, multifocal, or diffuse.  Most hepatic hemangiomas are of the cavernous type followed by the capillary type. Capillary hemangioma, including lobular capillary hemangioma (also known as pyogenic granuloma) commonly presents on the skin and mucosa, although rare liver or gastrointestinal tract examples have been reported. Capillary hemangioma is composed of a lobular proliferation of vascular channels with plump endothelial cells lining the vascular channels. Anastomosing hemangiomas in the liver, as seen in this case, are rare hepatic vascular neoplasms characterized by an interconnecting sinusoidal-like pattern of tightly packed capillary channels. The tumor appears well circumscribed grossly with a gray-brown focally spongy appearance in the liver. Low-power magnification shows a well-demarcated lesion with lobular architecture in the liver. At higher magnification the tumors consists of anastomosing sinusoidal capillary-sized vessels with scattered hobnail endothelial cells within a framework of non-endothelial supporting cells. No mitotic figures or necrosis are observed. Mild cytologic atypia is appreciated. Immunohistochemical stain for CD31 is diffusely positive.

The primary differential diagnosis of anastomosing hemangioma is angiosarcoma. Angiosarcoma of the liver and gastrointestinal tract is rare and behaves aggressively. The overall survival is poor and patients rapidly develop metastases and usually die within 6–12 months. Accurate diagnosis can be challenging, particularly if the patients have no history of exposure to specific toxins including thorium dioxide, arsenicals, and vinyl chloride monomer. Distinguishing anastomosing hemangioma from a well-differentiated angiosarcoma is challenging especially on small biopsies. Histologically, branching, jagged, slit-like vascular channels with prominent cytological alterations and a diffuse infiltrating border is characteristic of angiosarcoma, in contrast to the sharp demarcation, mild cytological atypia, and lack of mitotic figures in anastomosing hemangioma. The absence of multilayering of endothelial cells, high grade cytologic atypia, and mitotic activity coupled with circumscribed borders favor a benign process. Awareness of this entity and attention to cytological features and the overall lobular architecture are essential to avoid diagnostic errors.

In summary, anastomosing hemangiomas in the liver is a rare entity and leads to concern for angiosarcoma. Awareness of this entity can minimize a misdiagnosis of angiosarcoma and avoid unnecessary aggressive treatment.

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Contributed by:
Jingmei Lin, MD, PhD
Department of Pathology and Laboratory Medicine
Indiana University School of Medicine