A rare presentation of hepatic and splenic cystic malignant fibrous histiocytoma: A case report and literature review

Abstract

INTRODUCTION

Malignant fibrous histiocytoma is one of the most common soft tissue sarcomas in late adult life. But primary visceral malignant fibrous histiocytoma is a very rare entity. In peripheries, it is known to have an aggressive behavior but its biological pattern when involving liver and spleen is not well understood due to the rarity of its occurrence.

PRESENTATION OF CASE

A case of malignant fibrous histiocytoma of the liver and spleen as multiple cystic lesions in a 30 years old man is reported. The patient presented with hepatosplenomegaly resulting in central abdominal distention. Pre-operative investigations pointed toward the diagnosis of malignant cystic disease. The tumor presented as multiple hepatic cysts with massive hepatomegaly and splenomegaly. These cysts contained hemorrhagic fluid. Biopsy revealed highly cellular pleomorphic spindle cells fascicles arranged in storiform pattern at places with frequent mitoses. Immunohistochemistry revealed viamentin positivity. The tumor is compared with previous case reports.

DISCUSSION

Malignant fibrous histiocytoma of liver and spleen has been mentioned in the literature as isolated case reports and most of these present as solid lesions but presentation as multiple cysts is also a possibility as was seen in this patient. It can be confirmed only on histo-pathology supported by immunohistochemistry. The disease carries guarded prognosis due to its rapid progression and diagnostic dilemma pre-operatively.

CONCLUSION

This rare malignancy affecting the viscera can be diagnosed only with high index of suspicion and awareness regarding its presentation can help surgeons deal with it.

1. Introduction

Primary malignant fibrous histiocytoma of the liver and spleen is an extremely rare entity. In the literature, very few number of cases have been reported. This soft tissue sarcoma is known to occur in the extremities and trunk where it is known to have a very aggressive behavior. Its clinical and biological behavior when involving the liver and spleen is not well known because of the rarity of its occurrence. Previously, only a single case of splenic and hepatic malignant fibrous histiocytoma was reported that presented like multiple abscesses in the liver and spleen.⁷

This case report describes the presentation of this disease in a 30 years old man mimicking hepatic and splenic cysts.

2. Case report

A 30 years old man was admitted in the Department of General Surgery, Safdarjung Hospital, with complaints of pain upper abdomen, fullness in the abdomen from one and a half months, with no history of fever, weight loss, jaundice, vomiting, bladder and bowel disturbances. Prior to this presentation, he was healthy with no medical illness.

Physical examination was done which revealed distended abdomen, hepatomegaly and splenomegaly with ascites. No separate lump was palpable. There was mild jaundice. The chest and abdominal X-rays were unremarkable. Among the blood investigations, hemogram, blood glucose, kidney function tests, serum electrolytes were within the normal range. Liver function tests revealed total bilirubin 4.1 mg%, direct bilirubin 3.1 mg%, alkaline phosphatase 501 U/l. The prothrombin time was normal.

Abdominal ultrasonography revealed the following findings: liver grossly enlarged (18.9 cm) with multiple cystic lesions and echogenic debris seen in both hepatic lobes, spleen enlarged (20 cm) with similar cystic lesions with moderate ascites. The pancreas, gall bladder, bile duct, portal vein and kidney were normal. There was no evidence of any dilated bowel loops, mesenteric or retroperitoneal lymphadenopathy.

CECT abdomen was done which also supported the findings of ultrasound with diagnosis of cystic disease of the liver and spleen with multiple echogenic cysts probably malignant. Based on these clinico-radiological findings, a pre-operative diagnosis of malignant fibrous histiocytoma was difficult. A laparotomy was performed and the following findings were noted:

• •Massive hepatomegaly with multiple hepatic cysts in each of the hepatic lobes (Fig. 1) among which one of the left lobe cyst showed signs of calcification.
• •All the cysts on aspiration revealed hemorrhagic fluid (about 1 l of this fluid initially drained from all the cysts, Fig. 2).
• •Spleen massively enlarged with one large cyst measuring 12 cm × 12 cm. Cut section of the spleen showed solid and cystic areas (Fig. 3).
• •Moderate ascites was present.
• •No lymphadenopathy or no signs of metastases.

Fig. 1.

Photograph showing liver massively enlarged with multiple cystic lesions.

Fig. 2.

Photograph showing aspiration of hemorrhagic fluid from the hepatic cysts.

Fig. 3.

Photograph showing the cut section of the spleen with areas of necrosis, solid and cystic components.

Deroofing of the hepatic cysts along with splenectomy was done and the liver biopsy from the involved areas was taken and subjected to histopathology.

The patient initially did well but 3 weeks after the surgery worsened with derangement of liver function tests and coagulation profile. His condition deteriorated progressively and he died 3 weeks after the surgery.

3. Histopathology and immunohistochemistry

The biopsy revealed highly cellular picture with pleomorphic spindle cells fascicles arranged in storiform pattern at places. There were frequent mitoses (6–7/10HPF), extensive areas of necrosis. Occasional tumor giant cells and histiocyte like cells were seen which all were suggestive of malignant fibrous histiocytoma. Immunohistochemistry was done to confirm the diagnosis and it revealed the specimen to be viamentin and CD68 positive.

4. Discussion

This tumor involving the liver and spleen had the histological characteristics of malignant fibrous histiocytoma similar to that found in another case previously reported (Feng et al.).¹ Macroscopically, however, in contrast to the other cases reported previously which usually reported solid lesions, in our case the clinico-radiological findings supported the diagnosis of cystic disease pre-operatively. The origin of this tumor is still controversial. The presence of viamentin points to a mesenchymal origin.

The presenting symptoms of this patient were also non-specific and post-operatively he died after 3 weeks after surgery. Arends et al.² also reported a similar case of malignant fibrous histiocytoma of liver with post-operative mortality within 6 days. Conran and Stocker³ reported mortality 18 days after admission in their case report of hepatic malignant fibrous histiocytoma. Metastases was not found in our case although it has been reported in hepatic and splenic malignant fibrous histiocytoma by Wick et al.,⁴ Bruneton et al.,⁵ and Colovic et al.⁶

The occurrence of this disease is very rare and the course is very aggressive and therefore, the differential diagnosis of malignant fibrous histiocytoma has to be kept in mind in cases with pre-operative diagnosis of multiple cysts involving liver or spleen with high probability of malignancy.

Conflict of interest

No conflict of interest.

Funding

None.

Ethical approval

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Authors’ contributions’

AD assisted in the surgery and was involved in the preoperative work up and post operative care of the patient, conceived the write up and performed the literature search and manuscript preparation.

SVA performed the surgery, involved in the pre-operative work up and post operative care of the patient, helped in the preparation of the manuscript.

NK assisted in the surgery and was involved in the preoperative work up and post operative care of the patient.

GGG assisted in the surgery and was involved in the preoperative work up and post operative care of the patient.

DSK helped in the preparation of the manuscript and literature search.