Related Articles

Background

Malignant fibrous histiocytoma (MFH) is a common sarcoma affecting soft tissues of the body, especially of the extremities or trunk. Prognosis of the abdominal MFH is usually poor.

Case presentation

A 52-year-old female presented to our surgical outpatient clinic with a lower abdominal tumor that had been gradually increasing in size. Clinical examination revealed a firm, irregularly surfaced, fixed, painless, child-head-sized tumor located in her lower abdomen. Computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen revealed a polycystic tumor at the lower abdomen which was 15 × 13 × 11 cm in diameter and encased the colorectum to the left back side. A barium enema and a colonoscopy showed direct invasion to the rectum. In 2001, the tumor had been excised along with a low anterior resection of the rectum because of direct invasion. The origin of this tumor was the mesorectum. The weight of the excised tumor was 1,500 g, including 800 ml of a brown fluid. A histopathological diagnosis revealed a common type of MFH, in which mitotic figures are frequently seen.

Conclusion

This patient has survived without recurrence, for approximately 8 years since the completed tumor resection. It is important to obtain a complete resection during the MFH treatment.

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 transanal 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.

This article is a case report of a high grade, radio-induced, breast malignant fibrous histiocytoma (undifferentiated high grade pleomorphic sarcoma), which developed in a 44-year old female, seven years after breast conservative surgery and radiotherapy for a T1N0M0 invasive left breast ductal carcinoma. The sarcoma presented as a fast growing tumour, 9.5 cm in the largest diameter, with skin, left breast, chest wall muscle and rib invasion.

Neoadjuvant chemotherapy was performed with epirubicin and ifosfamide. Extended radical surgery according to oncological standards and soft tissue reconstruction were carried out. Despite bad prognostic features of high grade and large invasive sarcoma, the patient is currently, after 44 months of follow up, without local recurrence, or metastases, exceeding the 12.8-month mean recurrence period and mortality rate for these tumours larger than 8.1 cm (± 1.2 cm) as described in the literature.

Background

A malignant fibrous histiocytoma is a soft tissue tumor that most commonly occurs in the extremities, but rarely involves the liver. The clinical characteristics and therapeutic experiences of primary hepatic malignant fibrous histiocytoma are still limited.

Methods

Two cases of primary hepatic malignant fibrous histiocytoma were analyzed retrospectively, and all the literature concerning primary hepatic malignant fibrous histiocytoma was analyzed.

Results

In China, a total of 76 cases had been reported, among which 50 were men, with a male to female ratio of 1.9:1. Mean age of the patients was 51.0 years old, and more than 85 percent were older than 40 years. 82.9 percent (63/76) of hepatic MFH were solitary lesions, with tumor size ranging from 2.5 to 23.5 cm (average 10.3 cm). Major clinical presentation (78.4%) was abdominal pain or discomfort, accompanied with some other non-specific symptoms such as malaise, anorexia, weight loss, jaundice and fever, and small cases (14.9%) were asymptomatic. Computed tomography and ultrasound usually revealed the location of lesions. The rate of pre-operative misdiagnosis was extremely high, and 14.9 percent of patients were even misdiagnosed as a benign liver cyst, liver abscess or hematoma. Integrated resection was performed among the most cases (49/68), among which only a few ones (12 cases) were introduced to have no recurrence or metastasis or be still alive with no detail information provided, while among the cases with palliative operation or only a biopsy, the cases that were followed-up all died.

Conclusions

Hepatic malignant fibrous histiocytoma is a rare malignant mesenchymal tumor. The variable features of clinical presentations and images make the diagnosis difficult. Though the prognosis of primary hepatic malignant fibrous histiocytoma was rather poor, integrated resection might provide a few cases a good opportunity for surviving, suggesting that surgery might be an effective treatment.

Introduction

Malignant fibrous histiocytoma is a very common subtype of soft-tissue sarcoma in middle and late adulthood. However, malignant fibrous histiocytoma of the testis is very rare in adolescents.

Case presentation

We report here the case of a 14-year-old Han Chinese boy, who presented with left scrotal mass lasting for 20 days along with distending pain for 5 days. A physical examination revealed a chicken egg-sized, firm, well-defined mass and unclear epididymis. A B-scan ultrasonography of the left scrotum displayed a 9.0×5.2×4.5cm medium- or low-echoic lobulated mass, which suggested a left testicular neoplasm. A fine needle aspiration cytology examination revealed that the cells obtained from the patient’s testicular neoplasm were composed of myxoid spindle, and ovoid cells with nuclear atypia and mitotic activity, and arranged in a whirlpool or storiform pattern. Under histological examination, the tumor cells were arranged in a storiform pattern, which displayed mucoid matrix degeneration, and grew invasively. Consequently, a histopathological diagnosis suggested myxofibrosarcoma (or myxoid malignant fibrous histiocytoma).

Conclusions

An ultrasonic examination combined with fine needle aspiration cytology should be helpful for the initial differential diagnosis of testicular malignant fibrous histiocytoma. However, the final confirmation relies on histopathological examination. To the best of our knowledge, this is the first reported case of malignant fibrous histiocytoma of the testis in an adolescent.

Fibrous histiocytoma is a tumor composed of a mixture of fibroblastic and histiocytic cells. These lesions most often arise on the skin, but may rarely occur in soft deep tissues. The diagnosis of FH may be clinically difficult when the lesion is located in the deep tissues, and is frequently confirmed after local excision. The most important diagnostic necessity is the separation of this tumor from aggressive forms of fibrohistiocytic neoplasms. We present a unique case of malignant fibrous histiocytoma developing in left buccal mucosa very near to the surgical margin from which a benign fibrous histiocytoma of the mandible was excised 3 years earlier in a 31 year old female. Clinical and histopathological evidence were not sufficient to establish link between these two processes. This article aims to describe the case and discuss the findings.

Primary malignant fibrous histiocytoma (MFH) of the lung is extremely rare although it is among the most common soft tissue sarcomas in adults. Surgery is the primary mode of therapy, with high rates of local and distant recurrence, while radiation therapy appears to be a very useful adjunct, decreasing local relapse. We report a case of primary malignant fibrous histiocytoma of the lung. Fourteen years after surgical resection, the patient is still alive although with multiple metastatic lesions throughout his body.

Summary

Fibrous histiocytoma is a benign tumour composed of a mixture of fibroblastic and histiocytic cells. Based on the location of this tumour, fibrous histiocytoma are usually divided into cutaneous types and those involving deep tissues. These lesions most often arise on the skin, but may rarely occur in soft deep tissues. The diagnosis of fibrous histiocytoma may be clinically difficult when the lesion is located in the deep tissues, and is frequently confirmed after local excision. The most important diagnostic distinction is the separation of this tumour from aggressive forms of fibrohistiocytic neoplasms, including dermatofibrosarcoma protuberans and malignant fibrous histiocytoma. A 19-year-old male presented with a painless swelling on the right cheek. Detailed clinical and laboratory examinations were performed. The lesion had been totally excised under local anaesthesia, and histopathology revealed a benign fibrous histiocytoma. The diagnosis, location, treatment and prognosis of fibrous histiocytoma are also discussed.

Angiomatoid fibrous histiocytoma is an uncommon soft-tissue tumor of intermediate malignancy that is often misdiagnosed initially. As there is not one immunohistochemical marker that consequently stains positive or negative for angiomatoid fibrous histiocytoma, molecular diagnostics are becoming more widely used. So far three translocations have been reported to arise in angiomatoid fibrous histiocytoma: FUS-ATF1, EWSR1-CREB1, or EWSR1-ATF1. We present a case of angiomatoid fibrous histiocytoma on the upper arm of a 40-year-old female, which was initially misdiagnosed as metastatic melanoma in a lymph node. Revision of the pathology revealed an angiomatoid fibrous histiocytoma, which was later confirmed by a EWSR1-CREB1 translocation with molecular diagnostics. Furthermore, we review the relevant literature and provide an overview of all available case reports in the past ten years. This case report illustrates the importance for pathologists of knowing the typical pathology features of AFH and integrating immunohistochemical and molecular findings in order to prevent overdiagnosis of lymph node metastasis of a malignancy.

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.

Introduction

Calcifying fibrous pseudotumor is a rare benign soft-tissue lesion composed of fibrous tissue with abundant hyalinized collagen and dystrophic and often psammomatous calcifications. The cause of the disease is unclear but, usually, complete resection of the well-circumscribed tumor is sufficient to avoid recurrence of the disease. Here, we report an unusual case of this rare tumor that presented as two lobulated lesions in the calf muscle.

Case presentation

The patient was a 17-year-old Japanese girl who noted a hard mass in her left calf. Magnetic resonance imaging revealed two well-demarcated lobular masses in the soleus muscle, and the tumor was significantly enhanced by contrast medium. Preoperative differential diagnoses included soft-part tumors composed of fibrous tissue. However, making a definite diagnosis was impossible because a lobulated shape is rare for fibrous tumors. Biopsy demonstrated that the mass was a benign tumor composed of collagen-rich, hyalinized fibrosclerotic tissue. We performed marginal resection of the two nodules, including the fibrous tissue that connected them. Immunohistochemistry was positive for factor XIIIa and negative for anaplastic lymphoma kinase-1. These findings were helpful to distinguish calcifying fibrous pseudotumor from inflammatory myofibroblastic tumor. There was no sign of recurrence at 30 months after surgery.

Conclusion

To the best of our knowledge, this is the first case of bilobular calcifying fibrous pseudotumor that developed in an extremity. As described in the previous literature, simple excision was sufficient for the treatment of calcifying fibrous pseudotumor with two lobules.

Introduction

Malignant fibrous histiocytomas have been periodically reported as the primary tumor in various organs including the urinary bladder, and is the second most frequent sarcoma of the urinary tract in adults. This report discusses a case of the well established diagnosis of a malignant fibrous histiocytoma of the bladder occurring as a post-radiation cancer after the treatment of a cervical carcinoma. Our findings support those of many previous studies and make the view of the nature of the disease clearer.

Case presentation

We report the case of a 54-year-old Thai woman who had been treated with radiation therapy for cervical cancer, who presented to our facility with urinary incontinence. Initially, our patient was diagnosed as having a high-grade urothelial carcinoma. Subsequent radical surgery rendered the final pathological diagnosis, confirmed histologically and immunohistochemically as malignant fibrous histiocytoma, with clinical and pathological staging of T4b N0 M0. Adjuvant chemotherapy was provided for our patient.

Conclusions

This type of malignancy is very aggressive and easily misdiagnosed due to its rarity. Therefore, in a patient with a prior history of irradiation in the pelvic area, this should be considered as a differential diagnosis to ensure early correct diagnosis and treatment.

Background

Soft tissue sarcomas in the head and neck region are rare and often present a difficult differential diagnosis. The aim of our presentation is to point out the complexity of the diagnosis, treatment and follow up.

Case presentation

An eighty-seven year old female patient was referred to our unit with a fast growing brownish lump on the face. Four months beforehand, a benign fibrous histiocytoma (BFH) had been removed from the same location by excision biopsy with wide tumour-free resection margins. Excision biopsy of the recurrent lesion revealed a malignant fibrous histiocytoma (MFH). Radical tumour resection was completed by extended parotidectomy and neck dissection; the skin defect was covered by a regional bi-lobed flap. No adjuvant radio- or chemotherapy was administered. Full functional and cosmetic recovery was achieved; follow-up has been uneventful more than two years postoperatively.

Discussion

Malignant transformation of BFH is extremely rare and if so, extended radical surgery may give a fair chance for a favourable outcome even in patients with advanced age.

Malignant fibrous histiocytoma (MFH) is an uncommon soft-tissue sarcoma that occurs primarily in the extremities and rarely involves the retroperitoneum and abdomen. A 63-year-old man was admitted to the emergency room because of epigastric pain. Computed tomography revealed a large heterogeneous enhanced mass originating from the omentum with hemoperitoneum. The patient underwent laparoscopic omental mass excision and hematoma evacuation. Histological examination of the resected tumor revealed MFH. This case was therefore omental MFH presenting with hemoperitoneum.

Aneurysmal fibrous histiocytoma is a rare variant of cutaneous fibrous histiocytoma that results from blood vessel proliferation and haemorrhage into a fibrous histiocytoma. The resulting lesion has a very different clinical appearance, hence the potential confusion with other skin lesions. This report describes the case of a 48 year old woman with a recurrent fibrous histiocytoma with prominent vasculature, which over a three year period recurred on two occasions, showing more progressive features of the aneurysmal variant. In addition, squamous lined cysts were present within this tumour, a finding that has not been described previously. The histological features of this rare lesion and the importance of the differential diagnosis from other similar appearing malignant lesions will be discussed.

Malignant fibrous histiocytoma (MFH) of the small intestine is an extremely rare condition. It occurs most commonly in the extremities and the trunk. We report a case of a 67-year-old woman who admitted with fever, myalgia, and altered status. After thorough investigation, a tumor of the jejunum was found. The patient underwent complete surgical removal of the tumor. A diagnosis of MFN (undifferentiated high-grade pleomorphic sarcoma) was made. The patient received adjuvant chemotherapy with Gemcitabine. Two years after the operation, the patient died due to recurrence of the disease. MFH of the small intestine is an extremely rare neoplasm with an aggressive biological behaviour. In this paper, pathogenesis, natural history, and treatment are reviewed.

Malignant fibrous histiocytoma is a rare tumor with a poor prognosis. In experienced hands, laparoscopic splenectomy may be an appropriate choice for primary splenic sarcoma.

Background:

Splenic tumors are rare. Malignant fibrous histiocytoma (MFH) of the spleen is one of the least common primary splenic tumors. Review of the literature shows that a laparoscopic resection has never been tried.

Method:

We discuss the case of a 76-year-old man with a 7-cm MFH in the spleen and present a review of splenic sarcomas.

Results:

The patient underwent a successful laparoscopic splenectomy; pathology revealed a rare undifferentiated pleomorphic sarcoma of the spleen. A review of the international literature identified 15 additional cases of primary splenic MFH. Survival was rarely longer than 15 months.

Conclusion:

Malignant fibrous histiocytoma of the spleen is an exceedingly rare tumor with a poor prognosis. In experienced hands, laparoscopic splenectomy is a feasible operative choice for primary splenic sarcoma.

The occurrence of multiple primary malignancies (MPM) in one patient is a rare but increasingly frequent event, partly due to medical advances in diagnosis and therapy. A number of theories have been proposed to explain MPM, but none have been proven. A key risk factor appears to be family history. We present the case of a 43-year-old male with multiple cancers who was first admitted to the hospital for an undifferentiated pleomorphic sarcoma/pleomorphic malignant fibrous histiocytoma (pG2T2bN0Mx stage III) of the right scapula in May 2009. The patient underwent three tumor resections in situ due to tumor recurrence. During the period of chemotherapy and radiotherapy, computed tomography (CT) revealed a 10x10-cm enhancing soft mass of the ascending colon, which was diagnosed as mucinous adenocarcinoma in a pathological report. Laboratory data showed elevated serum levels of carcinoembryonic antigen (CEA, 20.0 μg/l; normal range, 0.0–10.0 μg/l). Certain family members of the patient had been diagnosed with endometrial, colon and pancreatic cancer. None of the family had a smoking history or presented with familial adenomatous polyposis (FAP). The patient with hereditary non-polyposis colorectal cancer (HNPCC), whose family fulfilled Amsterdam Criteria I (AC I), has remained free of disease for 15 months. Family history may be a key risk factor for MPM and HNPCC, the detailed molecular mechanisms of which remain to be elucidated. This case report with an investigation of family history may improve the clinical recognition of HNPCC and MPM.

Background

Primary visceral malignant fibrous histiocytoma (MFH) is a rare disease, and few cases have been reported in the English literature. However, retained foreign bodies in the abdomen after surgical procedures are important causes of intra-abdominal infections. For legal and ethical reasons, there are few publications in the literature. In this article, we describe for the first time a case of malign abdominal fibrous histiocytoma associated with a surgical sponge forgotten in the abdominal cavity a long time ago.

Case presentation

A 64-year-old male presented to our surgical department with cachexia, abdominal pain, distention and pyrexia of unknown origin. He had a medical history of abdominal surgery for peptic ulcer perforation 32 years ago. Clinical examination revealed fever with a distended and painful abdominal wall. Radiological imaging of the abdomen showed multiple heterogeneous masses in one large cystic cavityalmost completely filling the abdomen. The patient underwent a laparotomy, and interestingly, opening the cyst revealed retained surgical gauze (RSG). The origin of the tumor was the visceral peritoneum, and it was excised totally.

Conclusions

Primary intra-abdominal MFH can present as a complication of long-lasting RSG. Therefore, clinicians must remember this while establishing the differential diagnosis for patients with a history of previous abdominal surgery and presenting with symptoms associated with both the tumor and systemic inflammatory response.

Symptomatic mitral stenosis caused by a left atrial mass as the first sign of metastasis of a malignant tumor is extremely rare and frequently associated with poor prognosis. We report a case of a 59-year-old man with a history of grade 3 malignant fibrous histiocytoma on his left tigh treated by limb-sparing surgery 17 months earlier, who was admitted with 10-days of worsening dyspnea. Imaging revealed a left atrial mass protruding through the mitral valve that resulted in severe mitral stenosis. Biopsy confirmed metastasis of malignant fibrous histiocytoma.

INTRODUCTION

Fibrous histiocytomas are divided into two subgroups: malignant and benign fibrous histiocytomas (BFHs). BFH is one of the most common tumors of the superficial and deep soft tissues; it is commonly found on the skin and presents as a slow-growing solitary nodule made up of a mixture of fibroblastic and histiocytic cells.

PRESENTATION OF CASE

In this study, we present the case of a 45-year-old female who was histopathologically diagnosed with dermatofibrosarcoma protuberans (DFSP) and received radiotherapy, but whose diagnosis was changed to BFH based on subsequent immunohistochemical analyses.

DISCUSSION

BFH is a mesenchymal soft-tissue tumor with fibroblastic and histiocytic differentiation. Differential diagnosis for BFH found in deeper tissues includes other aggressive fibrohistiocytic lesions, such as DFSP and malignant fibrous histiocytoma. Differentiating among these tumors is crucial in selecting the correct surgical strategy and patient management in the postoperative period. In most cases, the pleomorphism and atypical mitotic activity seen histopathologically are sufficient for the differentiation between benign and malignant tumors. Immunohistochemical staining methods should be used in cases that are difficult to diagnose. The treatment of choice for BFH is wide resection of the tumor, which results in an excellent prognosis and low recurrence rate. In agreement with cases reported in the literature, our case confirms that wide excision is adequate to prevent the recurrence of the tumor.

CONCLUSION

Although benign fibrous histiocytomas is rare, it must be considered in the differential diagnosis of tumors arising from the soft-tissue.

Malignant fibrous histiocytoma is a sarcoma which commonly occurs in the soft tissues, joints and tendons of the extremities of adults but its occurrence in the oral and maxillofacial region is very rare. We present a rare case of malignant fibrous histiocytoma of the mandible with recurrence in the infratemporal fossa after surgery and radiotherapy. Patient underwent second surgery and complete tumor excision with minimal loss of function was achieved. Patient survived 8 years after surgery and died of natural cause.

Malignant fibrous histiocytoma (MFH) is an aggressive spindle cell cancer of soft-tissue sarcoma type in the elderly, mostly affecting the extremities. Lesions > 5 cm, positive margins, and local recurrence are significant poor prognostic indicators. The strongest predictor for distant metastasis was tumor size (> 5 cm), and for overall survival, presence of local recurrence. Limb-sparing extensive tumor resection is preferred to achieve negative surgical margins. However, in some circumstances, amputation is inevitable. Recent studies demonstrated that adjuvant radiotherapy for microscopically positive surgical margins significantly improved local control and disease-free survival rates. Therefore, effective therapeutic strategies against locally relapsed high grade MFH are required to prevent distant metastasis and to achieve long-term disease-free survival. Here, we report local relapse of high grade MFH treated by successive application of autologous formalin-fixed tumor vaccination (AFTV) with limb-sparing surgery and postoperative radiotherapy. The patient is alive and well, disease-free and with no functional impairment, more than five years after treatment.

Malignant fibrous histiocytoma (MFH) is a rare primary neoplasm that constitutes less than 1% of the malignant tumors of bone, and involvement of the skull is very rare. We present a case of malignant fibrous histiocytoma of the skull, presenting an intraosseous lesion in a 43-yr-old woman. She had a rapidly growing, tender mass in the right parietal region. A plain radiograph showed an osteolytic lesion of the right parietal bone. Magnetic resonance imaging revealed that the lesion showed heterogeneous low signal intensity on T1-weighted images and slightly high signal intensity on T2-weighted images. No evidence of an extraosseous extension to the adjacent dura and soft tissue was found, and a wide excision of the parietal bone was performed. Histologically, the tumor was a typical MFH displaying pleomorphic spindle cells in a storiform pattern. The results of immunohistochemical stainings revealed that the tumor cells were positive for vimentin, alpha-1-antitrypsin, and p53, and negative for smooth muscle actin, S100 protein, desmin, and MyoD1. Three months later, a mainly cystic, recurrent mass was developed at the previously operated site. Before the resection, we first performed the percutaneous aspiration cytology, revealing diagnostic multinucleated pleomorphic cells. Thereafter, she had to receive repetitive resections of recurrent or residual lesions, and she died of postoperative meningoencephalitis two years after the first operation.

Background

Fibromatosis or desmoid tumor covers a broad spectrum of benign fibrous tissue proliferations. It is characterized by infiltrative growth and a tendency towards recurrence; however, unlike sarcoma, it never metastasizes.

Case presentation

We report on a case of extraabdominal fibromatosis originating from the retroperitoneal space in a 43-year-old woman. Seven years earlier she had undergone ureterolysis and ureteroureterostomy for ureteral obstruction. Computed tomography revealed a tumor between the iliocostalis and the psoas muscle. Histopathological evaluation revealed uniform proliferation of spindle cells, with a moderate amount of collagen fibers, suggesting extraabdominal fibromatosis (desmoid tumor). The tumor was surgically resected, and since then, the patient has remained asymptomatic without any restrictions of daily living activities and without any signs of tumor recurrence during the two-year follow-up.

Conclusions

Complete resection is the treatment of choice. Adjuvant therapy using non steroidal anti-inflammatory agents, tamoxifen, interferon, anti-neoplastic agents, and radiotherapy, either alone or in combination finds application for unresectable or recurrent cases.