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Year : 2013  |  Volume : 9  |  Issue : 1  |  Page : 102-104

Intra-abdominal primary monophasic synovial sarcoma with hemangiopericytoma-like areas

1 Department of Pathology, Basic Sciences Block, Manipal, Karnataka, India
2 Department of Surgery, Kasturba Hospital, Madhav Nagar, Manipal University, Manipal, Karnataka, India

Date of Web Publication10-Apr-2013

Correspondence Address:
Padmapriya Jaiprakash
Department of Pathology, Basic Sciences Block, Kasturba Medical College, Madhav Nagar, Manipal University, Manipal, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.110393

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 > Abstract 

We report a case of retroperitoneal intra-abdominal primary monophasic synovial sarcoma (SS) with hemangiopericytomatous (HPC) pattern in a 25-year-old male arising from the triangular ligament on the superior surface of liver encasing the inferior vena cava (IVC) and masquerading as a hepatic tumor. A large heterogeneously enhancing, well defined, lobulated, exophytic lesion was seen involving segment VIII of the liver with foci of calcification in the periphery. A biopsy, followed by total resection of the tumor, showed a spindle cell sarcoma with HPC pattern, which was consistent with monophasic SS on histology and immunohistochemistry. The unusual clinical presentation, radiology, pathology, and differential diagnosis will be discussed in detail.

Keywords: Hemangiopericytoma-like, hepatoma, Intra-abdominal, sarcoma, synovial

How to cite this article:
Rao L, Jaiprakash P, Palankar N, Gowda V. Intra-abdominal primary monophasic synovial sarcoma with hemangiopericytoma-like areas . J Can Res Ther 2013;9:102-4

How to cite this URL:
Rao L, Jaiprakash P, Palankar N, Gowda V. Intra-abdominal primary monophasic synovial sarcoma with hemangiopericytoma-like areas . J Can Res Ther [serial online] 2013 [cited 2020 May 25];9:102-4. Available from: http://www.cancerjournal.net/text.asp?2013/9/1/102/110393

 > Introduction Top

Synovial sarcoma (SS) is a common soft tissue tumor of uncertain histogenesis commonly occurring in the deep soft tissues and para-articular regions of extremities. With the aid of immunohistochemistry and demonstration of the specific t(X;18) chromosomal translocation or resulting SYT/SSX fusion gene transcripts, this tumor has been described in most anatomical locations. Primary intra-abdominal SS is rare. [1] These aggressive tumors occur in either sex, mainly in middle age, and mostly arise in the retroperitoneum. Like other retroperitoneal sarcomas, they reach a large size before presentation and are difficult to excise and recur locally. [1] This is a case of primary intraabdominal retroperitoneal SS arising from the triangular ligament.

 > Case Report Top

A 25-year-old male presented with a 2 month history of nonradiating, dull-aching upper abdominal pain, without associated vomiting or altered bowel habit. Patient was a known alcoholic. Local examination revealed mild hepatomegaly. Liver function test revealed predominantly direct hyperbilirubinemia, elevated alanine and aspartate transaminase normal alkaline phosphatase, and alpha fetoprotein, mild increase in prothrombin time and activated partial thromboplastin time (PT and APTT). Computed tomography (CT) abdomen [Figure 1] revealed a large well defined lobulated exophytic mass measuring approximately 13.4×12.7×11.2 cm involving segment VIII which was hypodense on plain scan with peripheral foci of calcification and heterogenous enhancement in the arterial phase. Superiorly, the lesion was abutting the inferior surface of the right hemi-diaphragm. CT features were reported as suggestive of hepatocellular carcinoma. Diagnostic laparoscopy was done, the lesion biopsied and sent for histopathology.
Figure 1: CT image showing a well defined lobulated hypodense exophytic mass involving segment VIII with peripheral foci of calcification

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MICROSCOPY: Haematoxylin and eosin (H and E) stained sections showed a malignant spindle cell tumor with hemangiopericytomatous (HPC) pattern. Spindle cells were arranged in fascicular pattern with cells having indistinct cytoplasm, hyperchromatic nucleus and few mitoses, interspersed with numerous capillaries, along with large areas of necrosis and hemorrhage [Figure 2]. Reticulin fibers were abundant in the tumor. No liver tissue was identified. Immunohistochemically, the tumor cells were epithelial membrane antigen (EMA), CD99, and Bcl2 positive and negative for CD 117 and CD 34 [Figure 3]. S-100 was negative and pancytokeratin, CK7 and CK19 were done for confirmation, which showed focal positivity. However, Transduction-Like Enhancer1 Protein and fluorescent in-situ hybridization to detect translocation t (X;18) TLE1 and FISH study could not be conducted due to scarcity of resources. A histological diagnosis of primary intra-abdominal SS was rendered. Complete resection of tumor was then planned. Inferior vena cava(IVC) gram demonstrated the tumor encasing the IVC. The tumor was resected along with the IVC, which was then reconstructed. However, the patient suffered massive intra-operative blood loss and went in for hypovolemic shock and did not recover.
Figure 2: Fascicles of spindle cells with scant cytoplasm and hyperchromatic nuclei, arranged in hemangiocytomatous pattern. (H and E, ×100)

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Figure 3: Immunohistochemistry with occasional positivity for EMA, intense positivity for CD99 and bcl-2 and negative for CD34 with intervening endothelial cells being positive

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Grossly, a single unencapsulated mass weighing 840g and measuring 14×14×6 cm, with a defect (venous wall) was seen with grey white and hemorrhagic areas [Figure 4]. On cut section, gray white firm areas, focal yellow and hemorrhagic areas with cystic degeneration. Microscopy revealed similar findings as seen in the tru-cut biopsy. As the tumor was attached to and encasing IVC and was neither arising from the surface of liver or diaphragm, a diagnosis of primary SS arising from triangular ligament was rendered.
Figure 4: Unencapsulated mass with a defect in posterior wall, showing gray white firm areas with yellow and hemorrhagic foci

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 > Discussion Top

Primary retroperitoneal SSs are rare, representing about 1% of retroperitoneal tumors. Primary intra-abdominal SS are uncommon with some 33 examples mentioned in the literature, [1] of which, 11 have detailed clinical andpathological documentation. [1],[2] Few case reports have been documented, with a clinicopathological study of 11 cases by Fisher et al. [1] SS have been reported in virtually all anatomic sites including solid organs like the liver [3] and kidney. [4]

Morphologically, they are sub-classified into biphasic, monophasic (fibrous and epithelial), and poorly differentiated subtypes. The poorly differentiated SS may, in turn, show a large cell, small cell, or high-grade spindle cell morphology. [5] Focal HPC pattern of the vessels have been described along with myxoid change and cystic degeneration. [6],[7]

Intra-abdominal retroperitoneal monophasic SS can resemble a wide variety of other spindle cell neoplasms in the retroperitoneum, including adult fibrosarcoma, solitary fibrous tumor, [8] malignant peripheral nerve sheath tumor (MPNST), leiomyosarcoma, sarcomatoid mesothelioma, and extragastrointestinal stromal tumor (eGIST). [1],[9] Many SS have a HPC pattern leading to a misdiagnosis of hemangiopericytoma. [1] The latter is now regarded as a diagnosis of exclusion, and the presence of any differentiation marker indicates a more specific category. Appropriate use of immunohistochemical panels and genetic data can help in differentiating poorly differentiated SS from other small round cell sarcomas. [10] Poorly differentiated carcinomas can be excluded by a more diffuse cytokeratin pattern and identification of a primary site. [1]

In the present case, as the tumor cells were negative for CD 34, solitary fibrous tumor was ruled out. Negative staining for CD117, smooth muscle actin (SMA) and S-100, focal positivity for CK7 and CK19 ruled out eGIST, leiomyosarcoma, and MPNST, respectively. The tumor cells were EMA, Bcl2, and CD99 positive supporting the diagnosis of monophasic fibrous SS. In spite of extensive HPC pattern and positive CD99 stain, a diagnosis of malignant hemangiopericytoma was ruled out as the tumor cells were negative for CD34.

Immunohistochemistry has made a great contribution in the diagnosis of SS. Immunohistochemically, SS are positive for cytokeratin (either AE1/AE3 or CAM5.2), epithelial membrane antigen, vimentin, CD99, and calretinin. A significant number of cases are immunoreactive for bcl-2, calponin, Her2/neu and melanoma antigen family-C1 (Cancer testis)MAGE-CT. Keratin markers and EMA have been reported to react in 60-70% of the cases of monophasic SS while immunoreactivity for S100 protein has been reported in about 30% of the cases. [10] CD99 antigen [10] is of limited clinical use due to its nonspecificity. It has been recommended that most sensitive markers for the diagnosis of monophasic fibrous and poorly differentiated SS are EMA, cytokeratinAE1⁄AE3, and E-cadherin, with negative CD34 staining.

Recent studies have shown the usefulness of cytogenetic and molecular techniques as diagnostic adjuncts for distinguishing SS from other morphologically similar spindle cell sarcomas. [10]

Our case is unusual for the following reasons: (1) On CT, tumor appeared to arise either from the superior surface of liver or inferior surface of diaphragm. (2) On explorative laparotomy, the tumor was attached to the posterior surface of liver near the bare area and infiltrating the IVC and growing retroperitoneally. We, hence, postulate that the tumor in question is probably arising from the triangular ligament bordering the bare area of liver and infiltrating the IVC.

 > References Top

1.Fisher C, Folpe AL, Hashimoto H, Weiss SW. Intra-abdominal synovial sarcoma: A clinicopathological study. Histopathology 2004;45:245-53.  Back to cited text no. 1
2.Miyashita T, Imamura T, Ishikawa Y, Okinaga K, Kunii O, Miyashita H. Primary retroperitoneal synovial sarcoma. Intern Med 1994;33:692-6.  Back to cited text no. 2
3.Srivastava A, Nielsen PG, Cin PD, Rosenberg AE. Monophasic Synovial Sarcoma of the Liver. Arch Pathol Lab Med 2005;129:1047-9.  Back to cited text no. 3
4.Shannon BA, Murch A, Cohen RJ. Primary renal synovial sarcoma confirmed by cytogenetic analysis alesion distinct from sarcomatoid renal cell carcinoma. Arch Pathol Lab Med 2005;129:238-40.  Back to cited text no. 4
5.Weiss SW, Goldblum JR, editors. Malignant soft tissue tumors of uncertain type. Enzinger & Weiss's soft tissue tumors. China: Mosby Elsevier; 2008. p. 1161-82.  Back to cited text no. 5
6.Korula A, Shah A, Philip MA, Kuruvila K, Pradhip J, Pai MC, et al. Primary mediastinal synovial sarcoma with transdiaphragmatic extension presenting as a pericardial effusion. Singapore Med J 2009;50:e26-8.  Back to cited text no. 6
7.Eichelberger L, Jones TD, Vance G, Saxena R. A37-Year-Old Man With a Pleural Mass. Arch Pathol Lab Med 2005;129:1063-4.  Back to cited text no. 7
8.Chan JK.Solitary fibrous tumour-everywhere, and a diagnosis in vogue. Histopathology 1997;31:568-76.  Back to cited text no. 8
9.Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr NJ, Emory T, et al. Gastrointestinal stromal tumors¤smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunohistochemical study of 26 cases. Am J Surg Pathol 1999;23:1109-18.  Back to cited text no. 9
10.Pelmus M, Guillou L, Hostein I, Sierankowski G, Lussan C, Coindre JM. Monophasic fibrous and poorly differentiated synovial sarcoma: immunohistochemical reassessment of 60t(X;18)(SYT-SSX)-positive cases. Am J Surg Pathol 2002;26:1434-40.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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