|Year : 2012 | Volume
| Issue : 1 | Page : 117-119
Malignant fibrous histiocytoma of the spleen: An extremely rare entity
Leelavathi Dawson, Oneal Gupta, Ketan Garg
Department of Pathology, VMMC and Safdarjung Hospital, New Delhi, India
|Date of Web Publication||19-Apr-2012|
Sector III-A, H.NO.26, Ground Floor, Rachna Vaishali, Ghaziabad- 201010
Primary malignant fibrous histiocytoma (MFH) of the spleen is extremely rare. Since the first description of primary splenic MFH reported by Govoni et al in 1982, to the best of our knowledge, only twelve cases of MFH of the spleen have been reported in the literature. We herein report a rare case of primary splenic MFH in a 30-year-old Indian male who presented with abdominal pain with a history of recurrent hydatid cyst of liver and spleen. A computed tomography (CT) scan was performed and a diagnosis of splenic hydatid cyst was made. Splenectomy was done. On histopathological examination, a diagnosis of malignant mesenchymal tumor, possibly storiform variant of malignant fibrous histiocytoma, was made. On immunohistochemistry, the tumor was positive for vimentin and CD68. The post operative period was uneventful. Compared with the twelve previously cases of MFH of the spleen, our patient is the youngest case reported so far.
Keywords: Immunohistochemistry, malignant fibrous histiocytoma, spleen
|How to cite this article:|
Dawson L, Gupta O, Garg K. Malignant fibrous histiocytoma of the spleen: An extremely rare entity. J Can Res Ther 2012;8:117-9
| > Introduction|| |
Malignant fibrous histiocytoma (MFH) of the spleen is extremely rare. The first case was reported by Govoni  in 1982. Malignant fibrous histiocytoma (MFH) is a soft tissue sarcoma mainly occurring in the soft tissues, especially in the extremities and trunk, and other less common sites include the retroperitoneum, and the head and neck. It is an aggressive malignancy with high potential of local recurrence and distant metastases, and surgery with radical resection of the primary tumor with negative histological margins is the treatment of choice even with recurrence or metastasis.  Only twelve cases have been reported in the literature so far.  Because of its rarity, the clinical characteristics and biological potentials of MFH of the spleen are yet to be determined. 
| > Case Report|| |
A 30-year-old patient presented with complaints of pain of abdomen and swelling in the epigastrium for past four months. He had a history of recurrent hydatid cyst involving the spleen and the liver. On examination, he had no pallor, BP was 116/72 mm Hg and a swelling in the epigastrium was noted. Other hematological and biochemical investigations were within normal limits. Computed tomography (CT) revealed a single large cyst in the spleen with peripheral irregular enhancement. Multiple small cysts in the liver with internal hypo-attenuation, enhancing separate and ill-defined heterogeneous solid component were also identified. A diagnosis of splenic hydatid cysts was suggested. Splenectomy was performed and evacuation of necrotic material was done and the specimen was sent for histopathological examination. The post-operative period was uneventful.
Grossly, a specimen of spleen was received weighing 400 g and measuring 15 × 12 × 6 cm. On serial sectioning, a well demarcated grey white area was identified measuring 11 × 8 × 6 cm with central portion of the lesion showing necrosis [Figure 1]. Histopathological examination revealed an infiltrating highly cellular lesion with pleomorphic spindle cells arranged in intersecting fascicles and storiform pattern at places. Frequent mitoses and extensive areas of necrosis along with tumor giant cells and histiocyte like cells were also identified [Figure 2] and [Figure 3]. A diagnosis of malignant mesenchymal tumor, possibly storiform variant of malignant fibrous histiocytoma, was suggested. On immunohistochemistry, the tumor was positive for CD68 and vimentin [Figure 4]. The post operative period was uneventful. However, the patient did not report for a subsequent follow-up at our center.
|Figure 1: The resected specimen of spleen measuring 15 × 12 × 6 cm, with a mass measuring 11 × 8 × 6 cm along with necrosis|
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|Figure 2: Section reveals a highly cellular lesion with pleomorphic spindle cells arranged in intersecting fascicles and storiform pattern at places, with extensive areas of necrosis. The tumor and splenic stromal interface is also seen, (H&E stain, 200×)|
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|Figure 3: Section reveals pleomorphic spindle cells arranged in storiform pattern along with tumor giant cells and histiocyte like cells and frequent mitoses (inset), (H&E stain, 400×)|
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|Figure 4: Tumor cells showing immunoreactivity for vimentin [200×] and CD 68 [400×] IHC|
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| > Discussion|| |
MFH is the most common soft tissue sarcoma in the elderly individuals and men are more often affected than women. This malignant tumor mainly occurs in the lower and upper extremities, followed by the trunk and retroperitoneum in decreasing order of frequency. It is a frequently occurring soft tissue tumor with aggressive biological behavior. Primary MFH of the spleen is especially rare. , In fact, we were only able to find twelve cases of splenic MFH in the English-medical literature. Our patient is the youngest reported case so far making our report exceedingly rare. The incidence of splenic MFH in all primary spleen neoplasms is difficult to evaluate because the cases are distinctly rare. According to the cases reported, splenic MFH mainly occurs in the individuals aged from 40 to 60 years old, and there seems to be a slight male predominance. There is no typical clinical symptom, but abdominal pain, splenomegaly, weight loss and fever were the prominent features in about 70% of the patients. Ultrasonography (USG) and computed tomography (CT) are the two most valuable imaging techniques. However, neither USG nor CT is specific for MFH. There are no suggestive imaging patterns that can be used for the diagnosis prior to surgery. These tumor cells consist of histiocyte-like and fibroblast-like cells with multinucleated giant cells. , There are positive stainings for lysozyme, vimentin, CD68, and alfa-1 antitrypsin. ,,
MFH has been categorized into five types, based on the histopathologic subtype, including storiform-pleomorphic, myxoid, inflammatory, giant cell and angiomatoid variants. However, only the subtypes of storiform, pleomorphic and inflammatory variants have been reported in the MFH of the spleen in the previously reported literature. Multinucleated giant cells, foam cells and inflammatory cells are also usually observed. The accurate diagnosis of MFH depends on an accurate differential diagnosis from other sarcomas, observing karyomorphism and differential figures, and immunohistological staining. ,,
In our case, we could not get a definite preoperative diagnosis of the splenic tumor according to the physical examination and CT. The final diagnosis was confirmed by the results of the histopathology and immunohistochemistry. However, Katsuura et al,  have reported a case of MFH spleen diagnosed on contrast enhanced USG and which was further reported as inflammatory variant of MFH on histopathology.
Splenectomy is the treatment of choice, and the role of radiotherapy and chemotherapy as adjuvant therapeutic modality is not yet clear in retroperitoneal and visceral sarcomas.
| > Conclusion|| |
Primary sarcomas of the spleen are exceedingly rare with only ninety reported cases. MFH presenting as a splenic mass is even rarer with only twelve reported cases. It is an aggressive tumor with high potential to metastasize. The clinical symptoms of primary splenic MFH are usually nonspecific, such as abdominal pain, fever, weight loss, and splenomegaly are prominent features. Splenectomy including laparoscopic splenectomy with histological negative margins is the treatment of choice. The diagnosis is mainly confirmed by the histopathologist whereby immunohistochemistry and radiology aids to the diagnosis. Chemotherapy and radiotherapy are other therapeutic modalities for patients with metastasis, but the therapeutic effect is poor. The clinical behavior of splenic MFH needs to be studied in a larger number of patients with a longer follow-up.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]