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Pathologic Quiz Case: Elderly Man With Bright Red Blood Per Rectum.

A 71-year-old white man with no prior history of malignancy and a 2-week history of obstipation, increasing abdominal girth, and abdominal cramping sought medical attention after he began passing bright red blood per rectum. He underwent colonoscopy, which revealed a fungating and hemorrhagic rectosigmoid mass lesion. Shortly after colonoscopy, the patient experienced a rapid decrease in oxygen saturation and required intubation with mechanical respiration. During the next several days, he developed acute renal failure leading to multiple organ system failure and eventually death.

Before he died, the patient was sent for computed tomographic scans of the chest, abdomen, and pelvis, which revealed extensive "metastatic disease" involving the lungs, liver, and multiple bony sites. While in radiology, a computed tomographic scan-guided needle biopsy of a tumor nodule in the bony pelvis was conducted. The needle aspiration slides were relatively hypocellular and contained a mixture of discohesive cells. Some of these cells displayed overt cytologic features of malignancy, including large irregular nuclei and prominent eosinophilic nucleoli (Figure 1). Osteoclast-type giant cells were admixed with the malignant cells. The core biopsy demonstrated a sheetlike expanse of the same malignant cells, some of which possessed long, tapering cytoplasm. A smattering of acute inflammatory cells was also noted, and a few small capillary-sized blood vessels were apparent. The malignant cells were found to be focally strongly immunoreactive (positive) with cytokeratin AE1/3 (Figure 2). A diagnosis of metastatic sarcomatoid carcinoma was rendered based on the combined cytomorphology, histomorphology, and immunohistochemical data.

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At the time of autopsy, the patient was noted to have marked atherosclerotic disease and extensive metastatic spread of what was presumed to be a high-grade colonic carcinoma. Metastatic tumor was present in the bilateral lungs, liver, omentum, bones, bilateral adrenal glands, abdominal lymph nodes, and liver. The hepatic parenchyma was approximately 80% replaced by grossly necrotic and focally hemorrhagic tumor nodules with a large central mass measuring more than 10 cm in greatest dimension. The malignant tumor from the antemortem bone biopsy was found to be cytologically similar to the "metastatic tumor" seen in sections from the liver and other organs garnered from postmortem examination. The liver tumor, however, demonstrated a true "sinusoidal" pattern with interlacing channels of pleomorphic epithelioid cells arranged in ribbonlike whorls around erythrocytes (Figure 3). In addition to displaying focal immunoreactivity for cytokeratin, the malignant cells in the liver lesion were also found to display diffuse cytoplasmic immunoreactivity (positivity) for factor VIII and the vascular endothelial marker CD31 (Figure 4).

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What is your diagnosis?

Pathologic Diagnosis: Metastatic Hepatic Epithelioid Angiosarcoma

Angiosarcomas are relatively rare malignant neoplasms characterized by the formation of freely anastomosing vascular channels. They can occur in any region of the body, but the majority arise in the skin or superficial soft tissue, especially in the head and neck.[1] They have also been reported as primary neoplasms in numerous other sites, including breast, thyroid, heart, lung, pulmonary artery, liver, spleen, kidney, adrenal gland, uterus, ovary, vagina, testis, bone, and serous membranes. Angiosarcomas may develop in the setting of chronic lymphedema, after irradiation, or after exposure to vinyl chloride, thorium dioxide (Thorotrast), arsenic, anabolic steroids, or foreign bodies.[2] Angiosarcoma is the most frequent primary sarcoma of the liver. It is usually found in men in their sixth or seventh decade of life. The most common complaints at presentation in cases of hepatic angiosarcoma are abdominal pain, a palpable mass, or both. These tumors are highly aggressive, with most deaths related to hepatic failure, massive hemorrhage, or metastatic disease. The prognosis is poor despite therapy or excision, with a mean survival of 6 months.[3,4]

Angiosarcomas can be massive tumors and have a variable gross appearance, including hemorrhagic, solid, or cystic cut surfaces. The tumor may be diffusely infiltrative with indistinct borders, and small satellite nodules can also be present.[4] Microscopic examination reveals malignant cells invading liver parenchyma and vasoformative areas with vessels lined by malignant endothelial cells. Enlarged anaplastic tumor cells with nuclear hyperchromasia and high nuclear-cytoplasmic ratios line the sinusoids.[5] A variety of patterns may be displayed, including papillary, spindled, and epithelioid morphologies.[2] Cytologically, the individual cells have been reported to vary in appearance, ranging from slender tapering mesenchymal cells to rounded epithelioid cells with abundant cytoplasm, mimicking cells of adenocarcinoma. A variety of other findings have been described, including nuclear grooves, cytoplasmic vacuoles, and intracellular hemosiderin deposits.[2,6]

The tumor cells in angiosarcomas are generally immu noreactive with the Ulex europaeus lectin type i (UEA-1),[7-10] vimentin,[8,9,11] and with the endothelial markers factor VIII, CD34, and CD31.[2,4,7-11] Several descriptive series pertaining to patterns of immunohistochemical reactivity seen in angiosarcomas have reported positivity for cytokeratin.[2,7-11] In one study of 80 cases of angiosarcoma of soft tissue, cytokeratins of various molecular weights were detected in 35% of the cases, usually demonstrating focally strong immunoreactivity.[11] Some authors have indicated that cytokeratin-positive cells are seen more readily in poorly vasoformative areas of these neoplasms.[7]

In conclusion, we present a case of angiosarcoma of the liver, which at the time of presentation was widely metastatic. The disease process was incorrectly classified as a metastatic carcinoma by fine-needle aspiration and core biopsy, in part due to morphology and history and in part due to expression of cytokeratin. We share this case to point out that not every metastatic malignancy with cytokeratin immunoreactivity is a carcinoma.

References

[1.] Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF. Angiosarcoma: a report of 67 patients and a review of the literature. Cancer. 1996;77:2400-2406.

[2.] Boucher LD, Swanson PE, Stanley MW, Silverman JF, Raab SS, Geinsinger KR. Cytology of angiosarcoma: findings in fourteen fine-needle aspiration biopsy specimens and one pleural fluid specimen. Am J Clin Pathol. 2000;114:210-219.

[3.] Silverman JF, Geisinger KR. Fine needle aspiration cytology of the liver and pancreas. In: Silverberg SG, Delellis RA, Frable WJ, eds. Principles and Practice of Surgical Pathology and Cytopathology. 3rd ed. New York, NY: Churchill Livingstone; 1997:1964.

[4.] Ferrel LD. Surgical diseases of the liver. In: Silverberg SG, Delellis RA, Frable WJ, eds. Principles and Practice of Surgical Pathology and Cytopathology. 3rd ed. New York, NY: Churchill Livingstone; 1997:2014-2015.

[5.] Neshiwat LF, Friedland ML, Schorr-Lesnick B, Feldman S, Glucksman WJ, Russo RD. Hepatic angiosarcoma. Am J Med. 1992;93:219-222.

[6.] Wakely PE, Frable WJ, Kneisl JS. Aspiration cytopathology of epithelioid angiosarcoma. Cancer. 2000;90:245-251.

[7.] Ohsawa M, Naka N, Tomita Y, Kawamori D, Kanno H, Aozasa K. Use of immunohistochemical procedures in diagnosing angiosarcoma: evaluation of 98 cases. Cancer. 1995;75:2867-2874.

[8.] Gray MH, Rosenberg AE, Dickersin GR, Bhan AK. Cytokeratin expression in epithelioid vascular neoplasms. Hum Pathol. 1990;21:212-217.

[9.] Eusebi V, Carcangiu ML, Dina R, Rosai J. Keratin-positive epithelioid angiosarcoma of thyroid: a report of four cases. Am J Surg Pathol. 1990;14:737-747.

[10.] Wenig BM, Abbondanzo SL, Heffess CS. Epithelioid angiosarcoma of adrenal glands: a clinicopathologic study of nine cases with a discussion of the implications of finding "epithelial-specific" markers. Am J Surg Pathol. 1994;18: 62-73.

[11.] Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol. 1998;22:683-697.

Accepted for publication November 15, 2000.

From the Department of Pathology, Allegheny General Hospital, Pittsburgh, Pa.

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Article Details
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Title Annotation:metastatic hepatic epithelioid angiosarcoma
Author:Pereira, Telma C.; Vi, Linh H.; Prichard, Jeffrey W.; Sturgis, Charles D.
Publication:Archives of Pathology & Laboratory Medicine
Geographic Code:1USA
Date:Jul 1, 2001
Words:1228
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