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.
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.
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 (MFH) is a fairly common tumor in the deep soft tissues: the most frequent primary sites are the lower (49%) and upper (19%) limbs, but it has been reported even in the retroperitoneum and abdomen (16%), while localization in the breast is extremely rare (1-2). Breast cancer is rarely seen in males, accounts for approximately 1% of all breast cancer, and the breast sarcomas constitute less than 1% of breast tumors in both sexes. In the review of the literature, this is the third male and first young male with MFH. Here, we present a 37-years-old male patient who is diagnosed to have malignant fibrous histiocytoma in a variant of pleomorphic fusiform cell localized in the left breast. Following the wide local excision, the patient was given an adjuvant 50 Gy of external radiotherapy. He remained alive and well after 42 months of followup. We believe that reporting such few cases would contribute to forming treatment algorithms of rare tumors.
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.
Malignant fibrous histiocytoma; Anorectal junction; Transanal local excision
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.
Two cases of primary hepatic malignant fibrous histiocytoma were analyzed retrospectively, and all the literature concerning primary hepatic malignant fibrous histiocytoma was analyzed.
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.
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.
Malignant fibrous histiocytoma; Liver neoplasms; Diagnosis; Therapy
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.
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.
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).
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.
Testis; Malignant fibrous histiocytoma; Histopathology
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.
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.
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.
Liver; Malignant; Histiocytoma; Spleen; Storiform
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Benign tumour; Cheek; Benign Fibrous Histiocytoma
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.
Malignant fibrous histiocytoma; Omentum; Hemoperitoneum
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.
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.
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.
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.
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.
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.
Undifferentiated pleomorphic sarcoma; Malignant fibrous histiocytoma; Laparoscopic splenectomy
Malignant fibrous histiocytoma, a type of sarcoma, is a malignant neoplasm with uncertain origin that arises in both the soft tissues and the bone. The occurrence of primary malignant fibrous histiocytoma of the pleura is extremely rare. We report a case of a 65-year-old Korean man who is being diagnosed with primary malignant fibrous histiocytoma of the pleura.
Histiocytoma, Malignant Fibrous; Pleura; Surgery
Malignant fibrous histiocytoma (MFH) is a common soft tissue sarcoma usually involving limbs and retroperitoneum. MFH of the rectus abdominis muscle is extremely rare. Surgery in similar cases leads to large abdominal wall defects needing reconstruction. Biological and synthetic laminar absorbable prostheses are available for the repair of hernia defects in the abdominal wall. They share the important feature of being gradually degraded in the host, resulting the formation of a neotissue.
We herein report the case of an 84-year-old man with MFH of the rectus abdominis muscle which was resected and the large abdominal wall defect was successfully repaired with a biological mesh.
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.
multiple primary malignancies; hereditary nonpolyposis colorectal cancer; family history
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.
Benign fibrous histiocytoma; Malignant fibrous histiocytoma; Malignant change
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; Mandible; Infratemporal fossa
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor, with a low-grade malignant potential, occurring predominantly in children and young adults. Association between AFH and other malignancies has been rarely reported. A 27-year-old man who presented with a palpable abdominal mass was diagnosed as having testicular cancer with multiple liver and lung metastases. At 16 months after chemotherapy, a follow-up computed tomographic scan revealed a supraclavicular mass measuring 3 cm in size, which was suspected to be a recurrence. The patient underwent surgical excision, and the mass was pathologically diagnosed as a AFH. The patient has had no local recurrence and no distant metastasis for 12 months after resection. To the best of our knowledge, this is the first case report of AFH as a second tumor in a patient with testicular cancer.
Histiocytoma; Angiomatoid fibrous histiocytoma; Second primary neoplasms; Testicular neoplasms
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.
aneurysmal; dermatopathology; fibrous histiocytoma; squamous metaplasis
We report a rare case of recurrent retroperitoneal dedifferentiated liposarcoma with osteosarcomatous components. An 82-year-old male diagnosed with recurrent retroperitoneal liposarcoma underwent a tumor resection. Histologically, osseous matrix with osteoid and mature hyaline cartilaginous tissues with high cellularity were observed in a fibrous background through most of the tumor, and scattered MDM2- and CDK4-positive atypical hyperchromatic stromal cells were detected surrounding the dedifferentiated areas. Dedifferentiation occurs in up to 10% of well-differentiated liposarcomas, frequently resembling a malignant fibrous histiocytoma-like pleomorphic sarcoma. In contrast, divergent differentiation with osteosarcomatous components is considered to be extremely rare.
Dedifferentiated liposarcoma; osteosarcoma; retroperitoneum
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.
A benign fibrous histiocytoma (BFH) is one of the fibrohistiocytic groups of soft-tissue tumors for which spinal involvement is extremely rare. To the best of our knowledge, most spine-originating BFHs are bone tumors. We report the first case of BFH occurring in the intraspinal extradural space on the lumbar spine. A 66-year-old female presented with severe claudication symptom. The preoperative magnetic resonance images showed a huge intraspinal, extradural, thecal-sac-compressing soft-tissue tumor that extended along the right L5 root to the neural foramen. The tumor was a relatively well-marginated, inhomogeneous soft-tissue mass with some fluid-containing cystic portions that were well enhanced by the gadolinium contrast dye. After a total facectectomy, the tumor was removed completely. The patient had a good neurological recovery without complications, and no recurrence was noted at the 6-month follow-up.
Histiocytoma; Benign fibrous; Soft tissue neoplasms; Lumbar vertebrae