Osteoclastoma-Like Giant Cell Tumor of the Liver, an Extremely Rare Tumor

authors:

avatar Bita Geramizadeh 1 , 2 , * , avatar Kurosh Kazemi 3

MD, Department of Pathology
Transplant Research Center
MD, Hepatobiliary and liver Transplant Surgery

how to cite: Geramizadeh B, Kazemi K. Osteoclastoma-Like Giant Cell Tumor of the Liver, an Extremely Rare Tumor. Hepat Mon. 2017;17(9):e56097. https://doi.org/10.5812/hepatmon.56097.

Abstract

Giant cell rich hepatic tumors are divided into two types of mesenchymal and epithelial. Mesenchymal tumors are uncommon. Osteoclastoma-like giant cell tumor of the liver is an extremely rare mesenchymal hepatic tumor with very poor prognosis. To the best of our knowledge, only 5 cases have been reported in the English literature so far. Our case was a 64-year-old woman presented with abdominal pain. The patient was operated with the preoperative imaging diagnosis of angiosarcoma that after pathologic examination turned out to be osteoclastoma-like giant cell tumor. Unfortunately, the patient died shortly after surgery.

1. Introduction

Giant cell tumors are mesenchymal neoplasms primarily seen in bone and soft tissues; but they have also been rarely reported from other organs such as thyroid, gall bladder, and pancreas (1). Occurrence of this type of tumor in the liver is an extremely rare event and to the best of our knowledge, only 5 cases have been reported in the English literature (2-5). Herein, we report our experience with an aggressive giant cell rich non-epithelial mesenchymal tumor in the liver (osteoclastoma-like giant cell tumor) in a 64-year-old female as the 6th case in the English literature.

2. Case Report

A 64-year-old woman presented with abdominal pain. Her past medical history was unremarkable. Her physical examination was also unremarkable. The heart and the lung were normal. There was no hepatomegaly or splenomegaly in abdominal examination. The head and neck examinations were also normal.

Laboratory examination showed WBC = 9000/mL, Hb = 13 g/dL, and platelet = 250,000/mL. ALT, AST, GGT, and alkaline phosphates were all unremarkable. Tumor markers of CEA, CA125, and CA19-9 were all in normal range.

Abdominal sonography showed a liver mass. CT scan showed an enlarged liver with a large mass in the right lobe of the liver with irregular borders and central necrosis, measuring 20 cm in the greatest diameter. A few smaller lesions were also present. Portal vein thrombosis was also identified. Non-tumoral liver was unremarkable, meaning that there was no underlying disease in the non-tumoral liver parenchyma (Figure 1).

A Huge Mass in the Right Lobe of the Liver
A Huge Mass in the Right Lobe of the Liver

The patient was scheduled for surgery with the preoperative imaging diagnosis of angiosarcoma. In the operation, the mass was reported to be hypervascular by the surgeon and the surgeon’s diagnosis was angiosarcoma. Incisional biopsy was performed. The received specimen in the pathology lab was fragmented, brown, and full of blood, measuring totally 4X3X1 cm3.

Microscopic sections showed bloody background with many osteoclast-like giant cells intermingled with mononuclear cells (Figure 2A), which were reactive with CD68 and vimentin (Figure 2B) but nonreactive with all the epithelial markers. The histopathologic findings were very similar to giant cell tumor of the bone. Many mitotic figures were present in the atypical pleomorphic mononuclear cells, with high Ki67 (Figure 2C).

A, Histologic Sections Show Hemorrhagic Tissue with Many Giant Cells and Mononuclear Cells (H&EX250); B, IHC Sections Show Reactive Vimentin; C, IHC Sections Show High Ki67
A, Histologic Sections Show Hemorrhagic Tissue with Many Giant Cells and Mononuclear Cells (H&EX250); B, IHC Sections Show Reactive Vimentin; C, IHC Sections Show High Ki67

After surgery, the patient had an uneventful postoperative period; however, in less than 2 months she died of cardiorespiratory arrest.

3. Discussion

Osteoclastoma-like giant cell tumor of the liver is an extremely rare tumor in the liver so that only 5 cases have been reported in the English literature since 1980. Table 1 shows the characteristics of the above-mentioned 5 cases. The first case was reported by Munoz in 1980 (1). All of the reported patients including ours were older than 50 years. It does not seem to have any sex preferences, and it is equally distributed in females and males. All of the previously reported cases, except two, have been in the liver with no underlying disease. The first reported case in 1980 by Munoz was observed in a patient with alcoholic cirrhosis who presented symptoms of portal hypertension (1). The other case autopsied and reported by Zhang was also a cirrhotic patient with no definite cause (5).

Table 1.

Characteristics

AuthorYear ReportedAge, ySexClinical PresentationSize, cmOutcomeUnderlying liver disease
Munoz 1198087MPortal Hypertension11Died 32 days after operationAlcoholic Cirrhosis
Horie 2198766FHematoemesis; Nausea; Abdominal pain5Died in 42 days after operation-
Rudloff3200561FRight upper quadrant pain8Died after cardiorespiratory failure-
Bauditz4200654MIncidental7Died after 15 months-
Zhang5200665MRight upper quadrant pain13Died after 5 days Of hypovolemic shockCirrhosis
Current Case201764FAbdominal pain20Died after cardiorespiratory arrest-

The most common presenting symptom has been abdominal pain; however, as the table shows, one case was incidentally detected (4).

In all non-cirrhotic cases, liver function tests and tumor markers have been in normal range and therefore, seem to have no diagnostic value (1-5).

All of the reported tumors have been hemorrhagic, which has been misdiagnosed as angiosarcoma before pathologic diagnosis (2).

Microscopic examination has been the same in all the 6 cases including osteoclast-like giant cells with abundant eosinophilic cytoplasm and a variable number of nuclei, mixed with malignant pleomorphic mononuclear cells and hemorrhagic background. The most important point in histopathology of this tumor is the differential diagnosis of hepatocellular carcinoma rich in osteoclast like giant cells, which shows positive epithelial markers instead of mesenchymal (6). These tumors, which have most commonly been reported in pancreas, sometimes have typical areas of carcinoma (7).

All of the previously reported cases of osteoclastoma-like giant cell tumor of the liver expired shortly after surgery, indicating that the disease has a very poor prognosis.

References

  • 1.

    Munoz PA, Rao MS, Reddy JK. Osteoclastoma-like giant cell tumor of the liver. Cancer. 1980;46(4):771-9. [PubMed ID: 6994872].

  • 2.

    Horie Y, Hori T, Hirayama C, Hashimoto K, Yumoto T, Tanikawa K. Osteoclast-like giant cell tumor of the liver. Acta Pathol Jpn. 1987;37(8):1327-35. [PubMed ID: 3673575].

  • 3.

    Rudloff U, Gao ZQ, Fields S, Gecelter GR. Osteoclast-like giant cell tumor of the liver: a rare neoplasm with an aggressive clinical course. J Gastrointest Surg. 2005;9(2):207-14. [PubMed ID: 15694816]. https://doi.org/10.1016/j.gassur.2004.07.007.

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    Zhang S. Osteoclast-Like Giant Cell Tumor Of The Liver: A Case Report And Literature Review. Internet J Pathol. 2006;6:1-5.

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    Dahm HH. Immunohistochemical evaluation of a sarcomatoid hepatocellular carcinoma with osteoclastlike giant cells. Diagn Pathol. 2015;10:40. [PubMed ID: 25928039]. https://doi.org/10.1186/s13000-015-0274-4.

  • 7.

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