Primary malignant fibrous histiocytoma of the abdominal cavity:CT findings and pathological correlation

AIM:To study computed tomography(CT)features of abdominal malignant fibrous histiocytoma(MFH)in various rare locations.METHODS:We retroprospectively identified cases of MFH involving the abdominal cavity.Particular attention was paid to details regarding imaging features and histological types.RESULTS:The study population consisted of seven men and one woman,with a mean age of 52.5 years.Seven patients had some physical symptoms,while one was incidentally detected.The sites of origin were liver(n=3),greater omentum(n=1),superior mesentery(n=1),ileum(n=1),right psoas muscle(n=1)and right kidney(n=1).With the exception of the ileum lesion,all were of huge size.The contour of the lesions was more or less clear.Foci of necrosis were present in six lesions(n=6).On plain CT scan,all lesions were hypo to iso dense.The lesion in the greater omentum was cystic.One lesion(n=1)showed significant enhancement and the cystic lesion showed mild peripheral enhancement.An abundance of blood vessels surrounding the mass was seen in two lesions(n=2)and both were of the inflammatory variety.Pathological examination revealed storiform-pleomorphic variety(n=4),inflammatory variety(n=3)and myxoid variety(n=1).Two of the patients with inflammatory MFH had a clinical presentation of fever and one was afebrile,however,blood investigations in all three showed leukocytosis.CONCLUSION:Primary MFHs of the abdominal viscera and gastrointestinal tract are generally huge soft tissue masses containing areas of low attenuation and mild to moderate contrast enhancement.

A case of primary malignant fibrous histiocytoma of the pancreas: CT and MRI findings

Primary malignant fibrous histiocytoma (MFH) of the pancreas is rare and a distinct clinical entity. We report a case of recurrence of pancreatic MFH with computed tomography (CT) and magnetic resonance imaging (MRI) findings. A 67-year-old man presented with a history of decreased body weight over the past 6 mo. Abdominal CT revealed a large, multilocular cystic mass in the head of the pancreas with obvious atrophy in the body and tail of the pancreas. After 6 mo postoperatively, MRI demonstrated a recurrent large mass in the primary area of the head of the pancreas. The lesion was heterogeneous, hypointense to the liver on T1-weighted imaging, and heterogeneously hyperintense to the liver with a hypointense area in the central part of the tumor on fat-saturated T2-weighted imaging. Contrast- enhanced T1-weighted imaging demonstrated a large multilocular cystic mass with a cystic wall, fibrous septa and enhancement of solid components. To the best of our knowledge, this is the first report on recurrence of primary MFH of the pancreas, and the first with MRI findings.

Malignant solitary fibrous tumor involving the liver

Solitary fibrous tumors are predominantly benign and are most commonly found in the thoracic cavity and pleura; while reports exist in the literature of malignant solitary fibrous tumors and those located in extrathoracic organs, these cases are considered extremely rare. Herein, a case is reported of a malignant solitary fibrous tumor involving the liver that was diagnosed and treated in a 62-year-old woman. The patient presented with complaints of upper abdominal pain and unintentional weight loss. Computed tomography scan of the abdomen revealed a remarkably large mass, measuring 15 cm × 10 cm × 20 cm, which appeared to be unrelated to any particular organ. The intraoperative finding of a wide communication with the left liver suggested hepatic origin, and served as an indicator for tumor resection via left hemihepatectomy. The diagnosis of solitary fibrous tumor and its malignant nature was confirmed by histological and immunohistochemical examination of the resected tissues. Hepatic solitary fibrous tumor is very rare, and surgery remains the mainstay of treatment. Due to limited reports of such tumors in the literature, little can be said about the benefit of adjuvant therapy and prognosis for the rare cases with malignant histological findings.

Primary intestinal malignant fibrous histiocytoma:two case reports

Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine. Here we report two cases of primary intestinal MFH. The first case was a 70-year old man admitted for recurrent right lower quadrant abdominal pain. At laparotomy, a tumor was found originating from the cecum, with a suspicious metastatic nodule on the surface of the right lobe of the liver. A right hemicolectomy was performed followed by an ileotransverse end-to-end anastomotic reconstruction. The second case was a 43-year old man with intussusceptions of the small intestine. An emergent laparotomy revealed 4 pedunculated masses in the small bowel and a partial resection of the small intestine was performed. Though the symptoms were not typical, based on histological and immunohistochemical studies, the patients were diagnosed as MFH of the intestine. They were not treated with chemotherapy or radiotherapy and both died within 3 mo. MFH of the intestine is an extremely rare neoplasm with an aggressive biological behavior. The pathogenesis of this disease has not been clarified to date. Complete surgical excision is preferred, adjuvant chemotherapy or radiotherapy may be advisable.

Malignant fibrous histiocytoma presenting as hemoperitoneum mimicking hepatocellular carcinoma rupture

Malignant fibrous histiocytoma (MFH) is a pleomorphic mesenchynal sarcoma. It is uncommonly arises primarily from the intra-peritoneal cavity. Primary peritoneal MFH with tumor bleeding and rupture is rare. We describe the imaging features of a 70-year-old patient presenting with ruptured hemorrhagic peritoneal MFH at subhepatic area,accompanied by massive hemoperitoneum,mimicking a ruptured pedunculated hepatocellular carcinoma. Computed tomography (CT) revealed a large heterogeneous enhanced subhepatic mass with adjacent liver,gallbladder and colon invasion. Tumor hemorrhage and rupture complicated with peritoneal seeding and massive bloody ascites were also detected. Angiography showed a hypervascular tumor fed by enlarged right hepatic arteries,cystic artery and omental branches of gastroepiploic artery. The patient underwent laparotomy for tumor resection,but the tumor recurred one month after operation. To our knowledge,the CT appearance of ruptured intraperitoneal MFH complicated by hemoperitoneum has not been previously described.

Doege-Potter syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature

BACKGROUND Solitary fibrous tumor of the liver (SFTL) is a rare occurrence with a low number of cases reported in literature. SFTL is usually benign but, 10%-20% cases are reported to be malignant with a tendency to metastasize. The majority of malignant SFTL cases are associated with a paraneoplastic hypoglycaemia defined as Doege-Potter syndrome. Surgery is the best therapeutic treatment, however, long- life follow-up is recommended. CASE SUMMARY A 74-year-old man, was admitted to the emergency department after a syncopal episode with detection of hypoglycaemia resistant to medical treatment. The computed tomography revealed a solid mass measuring 15 cm of the left liver. An open left hepatectomy was performed with complete resection of tumor. Histopathological analyses confirmed a malignant SFTL. CONCLUSION Large series with long-term follow-up have not been published neither have clinical trials been undertaken. Consequently, the methodical long-term followup of surgically treated SFTLs is strongly recommended.

Intracranial malignant solitary fibrous tumor metastasized to the chest wall:A case report and review of literature

BACKGROUND Solitary fibrous tumor(SFT)is a rare fibroblastic mesenchymal neoplasm that affects spindle cell soft tissues with broad-spectrum biological behavior;it is predominantly benign,and rarely metastasizes.SFT occurs mainly in the tissue structure of the serosa in the pleura and the thorax,and can be found throughout the body,though extra-thoracic localization,including the cephalic region,is uncommon.We reported the first case of intracranial malignant SFT metastasized to the chest wall.CASE SUMMARY An 81-year-old Japanese man was referred to our hospital due to progressive gait disturbance and appetite loss.His medical history included partial resection due to brain tumor,four times,and 50-Gray radiation therapy at another hospital,starting when he was 74 years old.An unenhanced head computed tomography(CT)scan revealed an 8 cm×5.1 cm×6.5 cm mixed-density mass at the left frontal lobe,accompanying a midline shift,and an unenhanced chest-abdomen CT scan revealed a 6 cm×4.1 cm×6.5 cm low-density mass in the left chest wall.A CT-guided percutaneous lung biopsy was performed,and the pathological findings were SFT corresponding to brain tumor.Finally,the correct diagnosis of his brain tumor in history of past illness revealed to be SFT,and the unremovable tumor,namely present brain lesions enlarged and metastasized to the chest wall.We established a definitive diagnosis of intracranial malignant SFT metastasized to the chest wall.We notified him and his family of the disease,and offered palliative care.He passed away on the 29 th hospital day.CONCLUSION This case suggests the need for careful,detailed examination,and careful followup when encountering patients presenting with a mass.

Malignant solitary fibrous tumor of the pancreas with systemic metastasis: A case report and review of the literature

BACKGROUND Pancreatic solitary fibrous tumor(SFT) is a rare neoplasm of intermediate biological potential. So far, only 22 cases have been reported since 1999. All the cases, except one, exhibited benign features. Here, we report the first case of malignant pancreatic SFT with typical Doege-Potter syndrome, along with the clinical and pathologic evidence of its systemic metastasis.CASE SUMMARY The patient was a 48-year-old man with a 1-year history of pancreatic and liver masses and refractory hypoglycemia. Increased uptake of the tracer fluorodeoxyglucose(FDG) was found in the liver and bones by fluorine-18 FDG positron emission tomography/computed tomography. After multidisciplinary discussion, a distal pancreatectomy procedure was performed, and histological examination showed a lesion composed of abundant heterogeneous spindle cells with localized necrosis. On immunohistochemistry evaluation, STAT6 was found to be diffusely expressed in the tumor. Based on the overall evidence, the patient was diagnosed with malignant pancreatic SFT with liver and bone metastases.CONCLUSION The diagnosis of malignant SFT requires comprehensive evidence including clinical, immunohistochemistry, and histological features. This case may be presented as a reference for diagnoses and management of malignant pancreatic SFTs with systemic metastasis.

Obstructive jaundice caused by secondary pancreatic tumor from malignant solitary fibrous tumor of pleura:A case report

A 77-year-old man on systemic chemotherapy against postoperative bilateral multiple lung metastases of malignant solitary fibrous tumor of the pleura suffered from pruritus and jaundice. Blood examination showed elevated levels of hepatobiliary enzymes. Abdominal computed tomography showed a tumor with peripheral enhancement in the pancreatic head, accompanied with the dilatation of intra- and extra-hepatic bile ducts. He was diagnosed as having obstructive jaundice caused by a pancreatic head tumor. The pancreatic head tumor was presumably diagnosed as the metastasis of malignant solitary fibrous tumor of the pleura, because the findings on the pancreatic head tumor on abdominal CT were similar to those on the primary lung lesion of malignant solitary fibrous tumor of the pleura. The pancreatic tumor grew rapidly after the implantation of metallic stent in the inferior part of the common bile duct. The patient died of lymphangitis carcinomatosa of the lungs. Autopsy revealed a tumor that spread from the pancreatic head to the hepatic hilum. Microscopically, spindle-shaped cells exhibiting nuclear atypicality or division together with collagen deposition were observed. Immunohistochemically the pancreatic head tumor cells were negative for staining of α-smooth muscle actin （α-SMA） or CD117, but positive for vimentin, CD34 and CD99. These findings are consistent with those on malignant solitary fibrous tumor of the pleura. We report the first case of obstructive jaundice caused by a secondary pancreatic tumor from malignant solitary fibrous tumor of the pleura.

Simultaneous occurrence of malignant fibrous histiocytoma and hepatocellular carcinoma in cirrhotic liver: A case report

Primary hepatic malignant fibrous histiocytoma (MFH) is rarely encountered. There have been no reports to date of hepatic MFH associated with liver cirrhosis. The presence of liver cirrhosis is considered an adjunctive feature favoring sarcomatoid hepatocellular carcinoma (HCC) in the diagnosis of spindle cell tumors in liver. We describe here a 59-year-old man with liver cirrhosis due to hepatitis B virus infection 20 years ago. On abdominal computed tomography scanning, two distinct hepatic masses were identified in the background of cirrhosis, which had different radiological features from conventional HCC. He underwent segmentectomy for removal of the tumors. The pathological examination of surgically resected specimen revealed the large malignant spindle cell tumor and small conventional HCC. Additional tissue sampling and immunohistochemical stainings demonstrated that the spindle cell tumor was consistent with MFH. On the post-operative follow-up for 21 mo, a round mass showing similar radiological findings for the previous MFH was appeared on the surface of resection margin, suggesting the recurrence. Despite its rarity, hepatic MFH should be considered during differential diagnosis, even in cirrhotic patients, and extensive tissue sampling and immunohistochemical analyses are necessary in the diagnosis of hepatic spindle cell tumors.

Malignant fibrous histiocytoma of the bone in a traumatic amputation stump: A case report and review of the literature

BACKGROUND Malignant fibrous histiocytoma(MFH)is one of the most common soft tissue sarcomas among adults.It is characterized by large size,high grade,and biological aggressiveness.There are many reports of MFH after local stimulation,such as bone fracture,implants,and chronic osteomyelitis.In this paper,we report a patient who developed MFH 6 years after amputation,suggesting that wound healing and mechanical force play a role in the local stimulation of this disease.CASE SUMMARY A 66-year-old man complained of persistent pain in his residual mid-thigh.He had undergone amputation surgery due to a traffic accident 6 years prior.Physical examination showed tenderness but no abnormalities in appearance.Xray radiographs and magnetic resonance imaging supported the diagnosis of a tumor,and a biopsy confirmed that the lesion was MFH.The patient received neoadjuvant chemotherapy and left hip disarticulation.During the 6-mo followup,there were no symptoms of recurrence.CONCLUSION Postsurgery MFH has been reported before,and many studies have attributed it to the biological effects of implants.Our case report shows that this disease can develop without an implant and thus highlights the importance of local stimulation.The wound-healing process and mechanical force can both promote this tumor,but whether they directly cause MFH needs further investigation.

Malignant solitary fibrous tumor of the greater omentum: A case report and review of literature

BACKGROUND Malignant solitary fibrous tumors(SFTs)account for 15%-20%of all SFTs,and malignant SFTs arising from the greater omentum are extremely rare.Most malignant SFTs of the greater omentum are diagnosed via pathological examinations after surgery.In this study,we report a case of malignant omental SFT and review the published literature on this rare malignancy.CASE SUMMARY A 64-year-old female presented with an abdominal mass,and underwent exploratory surgery,during which a huge tumor originating from the greater omentum and intraperitoneal implants were identified and resected.The results of the pathological examination,immunohistochemistry staining,and gene sequencing led to the diagnosis of malignant SFT of the greater omentum.The patient died one and a half years later due to tumor recurrence and metastasis.CONCLUSION This is the first report of the application of gene sequencing in the diagnosis of malignant SFTs of the greater omentum.

A case of malignant fibrous histiocytoma of the head after trauma and radiation therapy

Malignant fibrous histiocytoma(MFH) firstly described as"malignant fibrous xanthoma"by O' Brien and Stout in 1964, is the most common soft tissue sarcoma of late adult life.Uncertain histogenesis and numerous subtypes make MFH a rather controversial entity.MFH only rare arises from structures of the head and neck.When it does, it most often originates in facial structures, particularly the maxilla.This report details a case of a patient with malignant fibrous histiocytoma presenting clinically as a right-sided large indurated frontoparietal mass, three months after head trauma and eight years after radiation therapy for brain lymphoma located in the right frontal and parietal lobes.Radical excision was a surgical challenge because of the extensiveness of the lesion.

Transanal excision of a malignant fibrous histiocytoma of anal canal:A case report and literature review

Malignant fibrous histiocytoma, which is composed of spindle-shaped cells arranged in a pleomorphic and storiform pattern, is rarely found in the colorectum. Although complete surgical excision remains the main stem of therapy, an optimal treatment strategy according to the stage has not been elucidated. We report a case of a 63-year-old woman with an ulcerative lesion in the anorectal junction and a final diagnosis of malignant fibrous histiocytoma. We introduced an access for transanal local excision and adjuvant radiotherapy because the patient refused abdominoperineal resection. No local recurrences or distant metastases were observed 15 mo after the operation. To our knowledge, this is the first case reported in the English literature of a malignant fibrous histiocytoma treated with the transana local excision and adjuvant radiotherapy. This report showed that this approach is selectively reserved for early-stage malignant fibrous histiocytoma and for those patients who refuse radical surgery because of the risk in a permanent colostomy.

Malignant fibrous histiocytoma of the axilla with breast cancer:A case report

BACKGROUND Multiple primary malignant neoplasms refer to multiple tumors with different origins.They may be synchronous or metachronous.The incidence is 0.73%–11.7%.Synchronous cases of breast cancer with sarcoma are rare.CASE SUMMARY Here,we report a 78-year-old female patient admitted to hospital after accidental discovery of a left axillary mass.Preoperative examination revealed a breast mass.Pathology showed left breast cancer and left axillary sarcoma.The patient underwent surgery,endocrine therapy and radiotherapy.She has been followed up for 1 year,and no local recurrence or distant metastasis was observed.CONCLUSION Attention should be paid to multiple primary malignant neoplasms,not limited to the current diagnosis and analysis,avoiding missed diagnosis and misdiagnosis.

Pancreaticoduodenectomy for Pancreatic Metastases of Malignant Fibrous Histiocytoma

Unlike primary pancreatic carcinoma, metastases to the pancreas are rare and their resection may be performed as a palliative treatment due to poorly defined outcome. We herein present an extremely rare case of pancreatic metastases of malignant fibrous histiocytoma (MFH) undergoing pancreaticoduodenectomy with tumor-free survival within postoperative 35-month follow-up. Pancreatic resection for metastatic MFH to the pancreas should be considered in selected patients. Long-term survival or good palliation may be achieved.

Primary malignant fibrous histiocytoma of the esophagus: Report of three cases and review of the literatures

We reported three cases of polypoid tumor of the esophagus, among them one case of sarcomatous tumor partly covered with superficial squamous cell carcinoma. The sarcoma was consisted of anaplastic spindle and pleomorphic tumor cells, which was similar to malignant fibrous histiocytoma (MFH) of the soft tissue. Diagnosis of the surgery resected speci-men was confirmed by histological, immunohistochemical and electron microscopic methods. Both diagnostic and differential diagnostic problems of primary MFH of the esophagus and world medical literatures were discussed.

A case of primary malignant fibrous histiocytoma of the pancreas with liver metastasis

Malignant fibrous histiocytoma(MFH) is one of most common types of soft-tissue sarcoma. However, it accounts for less than 1% of all human cancer types. In addition, primary MFH of the pancreas is very rare. Herein, we report of a 37-year-old man who presented with a pancreatic mass and did not show any specific symptoms. Pre-contrast computed tomography(CT) showed a heterogeneous, liquid-filled necrotic mass in the uncinate lobe of the pancreas. Contrast-enhanced CT showed a mild enhancement of solid components and the pancreaticoduodenal artery across the mass. The patient underwent a pancreaticoduodenectomy, ethanol ablation of the liver lesions, and chemotherapy with 4 cycles of ifosfamide and doxorubicin. The tumor recurred in the liver, but not in the pancreas, after 8 months.

Early Recurrence of a Cardiac Malignant Fibrous Histiocytoma

Malignant fibrous histiocytoma (MFH) is a rare tumor of the heart and the patients with these tumors usually have a poor prognosis. We report a case of MFH with an origin from the left superior pulmonary vein, involving the left atrium and protruding through the mitral valve, which needed urgent surgery. Complete resection was performed but local recurrence was detected one month later.