Difficulties in diagnosing angiomatoid fibrous histiocytoma of the head and neck region:A case report

BACKGROUND Angiomatoid fibrous histiocytoma(AFH)is a rare,slow-growing soft tissue tumor.It appears mostly on the limbs and trunk in children and young adults.The biology of AFH remains unclear because of the small number of reported cases.Diagnostic testing does not provide definitive results.It has two clinical forms,that differ in terms of gene expression and clinical prognosis.It is important to inform the laboratory which specific gene testing is necessary.Here,we describe a case of rare AFH in the submandibular region using a full genetic panel.CASE SUMMARY A 13-year-old boy who had been misdiagnosed in the past 6 mo by his dentist visited our clinic because of a lesion in the submandibular area on the right side.The lesion was homogeneous and painless upon palpation.No skin discoloration was observed.Due to the non-specific radiological picture computed tomography(CT),magnetic resonance imaging(MRI),cone-beam CT(CBCT),and ultrasoundguided biopsy were performed.A venous malformation was suspected on the MRI.None of the tests provided a definitive diagnosis.Owing to the non-specific radiological findings,the patient qualified for surgical treatment.The surgical procedure included an excisional biopsy.The diagnostic testing was extended using gene rearrangements.The most distinctive gene translocation in diagnosing AFH is within the EWS RNA-binding protein 1(EWSR1)-CREB-binding protein.However,in this case,the diagnosis was confirmed by a rearrangement within the EWSR1 gene testing.CONCLUSION AFH in the submandibular location is rare,and surgical treatment with genetic evaluation defines AFH type that affects subsequent procedures.

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.

Hsp70 confines tumor progression of rat histiocytoma and impedes the cytotoxicity induced by natural killer cells and peritoneal macrophages

Objective:To study the role of inducible form of heat shock protein 70(Hsp70) in the host tumor regression of rat tumor model.Methods:We examined the role of Hsp70 in host tumorigenicity and in vitro cellular cytotoxicity using a rat histocytoma.The differential tumor growth and regression kinetics were studied and correlated with the expression of Hsp70,activation of macrophages and natural killer(NK) cells,and circulating or tumor infiltrating immune molecules in the host system.Results:The sub cuteaneous(s.c.) tumor regression was correlated with increased serum cytokines such as IL-12,TNF P,IFNγand Hsp70.Despite of similar increase of Hsp70 in intraperitoneal(i.p.) tumor implanted animals,animals succumb to tumor growth,further,evidently,no immune molecule activation was observed.The viral promoter driven Hsp70 over expression in these tumor cells restrained solid tumor growth,however,failed to inhibit ascites growth.The NK cells from s.c.immunized animals induces cytotoxicity in the presence of anti-tumor antibody,which necessitated CD40-L expression,conversely,NK cells from i.p.immunized animals failed to induce cytotoxicity.The NK cells from s.c.or i.p.implanted animals with Hsp70 positive tumor cells failed to induce such cytotoxicity.The peritoneal macrophages isolated from s.c.tumor implanted animals when co-cultured with parental BC-8 cells lyses tumor cells,nevertheless entail macrophage specific TNFαexpression.On the contrary,Hsp70 expressing BC-8 tumor cells were resistant to peritoneal macrophage induced cytolysis.Conclusions:This study brings out that Hsp70 possibly involved in regulating the host tumor response and cellular cytotoxicity.

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.

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.

Aneurysmal Fibrous Histiocytoma:Clinicopathology Analysis of 30 Cases of a Rare Variant of Cutaneous Fibrohistiocytoma

Aneurysmal fibrous histiocytoma is often clinically misdiagnosed.In this study,we put forward an insight on how to help diagnose this disease clinically.A retrospective chart review was performed on all patients diagnosed with aneuiysmal fibrous histiocytoma from 2007 to 2017 in the Department of Dermatology,Union Hospital,China,and all clinical data were collected from the hospital archives.From a total of 418 patients diagnosed with cutaneous fibrous histiocytoma,only 30 patients were confirmed to have aneurysmal fibrous histiocytoma out of which only 2 patients were clinically diagnosed with aneurysmal fibrous histiocytoma.The remaining 28 patients were diagnosed with various types of vascular tumors although pathology classified them as having aneurysmal fibrous histiocytoma.Among the 30 patients,9 were male and 21 were female.There were following age groups:13-19(mean 16,n=4),20-29(mean 26.25,n=8),30-39(mean 33,n=7),40-49(mean 44,n=4),50-59(mean 56.75,n=4)5 60 and above(mean 61,n=3).Tumors were present on the head,neck,back,waist,hips and upper and lower extremities.After complete excision,there was no recurrence and no complications.Histologically,lesions showed the typical pseudoangiomatoid spaces without endothelial lining and infiltration of fibrohistiocytes in hemosiderotic pigmentation.It was suggested that although the prognosis of aneurysmal fibrous histiocytoma is good,accurate diagnosis is paramount to avoid clinical misdiagnosis and subsequent complications.

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.

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.

DETECTION OF POINT MUTATION OF p53 GENE BY SILVER STAINING PCR-SSCP IN PARAFFIN-EMBEDDED MALIGNANT FIBROUS HISTIOCYTOMA

Silver staining PCR-SSCP method was used to detect point mutation of p53 gene in paraffin-embedded malignant fibrous histiocytoma (MFH) tissues. The abnormal shifting of the single-stranded DNA (ssDNA) was identified in 9 out of 16 cases (56.3%). The positive figure of SSCP was 1, 4, 4, 3 in exon 5, 6, 7, 8, respectively. The mutant of p53 protein was detected by microwave oven treatment and ABC immunohistochemistry. Positive nuclear staining was observed in 10 cases (62.5%). The positive coincidence rate was 90.0% between SSCP and p53 protein expression. The mutation of p53 gene was not correlated with the subtypes of MFH. Our results indicate that detection of point mutations with silver staining PCR-SSCP is convenient, rapid and reliable in the screening of point mutation of genes.

CT FINDINGS OF MALIGNANT FIBROUS HISTIOCYTOMA

Purpose: To define the sites and CT findings of 45 MFH Patients. Materials and Methods: The primary sites were:cranio-facial 16, trunk and extremity 12 and abdominal 17 (13 retroperitoneal). As seen on CT scans, the lesion is clear-margined and even when small, but, when large (42/50,>5 cm), it is uneven in consistency and apt to invade the nearby organs (75.8%). The CT Findings of untreated and recurrent MFH are similar. Results:Enhanced CT scans may provide useful information such as medium to hyper-attenuation (78.8%), necrosis (60.6%)or involvement of para-nasal sinuses as expanding deformity (8/9 cases). Conclusions: CT scan is important to delineate the extent of MFH Iesions of which the diagnosis is best ascertained by combining CT scan and histopathology. Enhanced CT scans can reveal much useful information.

Clinic Pathological Study of Aneurysmal Fibrous Histiocytoma

Aneurysmal fibrous histiocytoma (AFH) is a rare variant of benign fibrous histiocytoma (FH), Characterized by blood-filled spaces within the fibrohistiocytic tumor. AFH has a higher recurrence rate than FH. The diagnosis of AFH is often problematic due to its overlapping morphological features with other skin tumors. The diagnosis of AFH depends on histological features and immunohistochemistry. The aim of this study is to understand the clinical and histopathological diagnostic criteria for AFH.

P53 pseudogene: potential role in heat shock induced apoptosis in a rat histiocytoma

The p53 tumor suppressor gene is either non- functional or highly and frequently mutated in majority of cancers. In our study towards understanding cellular adaptations to stress using a rat histiocytic tumor model, we have identified mis-sense mutation in p53 that led to premature termination of translation at the carboxyl-termi- nus. Further, the cDNA isolated from heat stre- ssed cells producing two amplicons with cDNA specific primers (N-terminus) suggested occurrence of possible pseudogene(s). A comparative analysis between different tumor cell lines of rat origin and rat genomic DNA using p53 gene specific primers resulted in the amplification of a processed pseudogene and its positive interaction with wild type p53 probe on Southern blot analysis. The genomic DNA sequence analysis, and sequence comparison with cDNA discovered that the processed pseudogene lacks DNA binding domain and nuclear localization signal, however, contains the ribosomal entry and stop signals. Rat genome BLAST analysis of the pesudogene suggested chromosome-18 localization which was in addition to 14, 13, 10, 9 localization of the cDNA. In the interest of unraveling hidden dimensions of p53 tumor suppressor gene, our study explores the probability of p53 functional pseudogenes in rat histiocytoma.

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.

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.

Benign Fibrous Histiocytoma of the Neurocranium

Presented is a case of benign fibrous histiocytoma (BFH) involving the calvarium of a 25 years old lady who noticed a depression in her occiput associated with localised pain. Imaging revealed a tumour eroding through the inner and outer skull tables, closely associated with major underlying dural sinuses. She underwent complete macroscopic resection of the tumour and reconstruction of a titanium mesh cranioplasty. Histology favoured a benign process with a diagnosis of BFH of the calvarium given. At 1 year follow-up, the patient is asymptomatic and has not developed recurrence of the tumour.

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.

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.