Malignant fibrous histiocytoma of the glans penis: a case report

BACKGROUND: Malignant fibrous histiocytoma has been regarded as the most common sarcoma of older adults. However, recent opinion regards pleomorphic malignant fibrous histiocytoma as an undifferentiated high grade pleomorphic sarcoma not otherwise classifiable utilizing current techniques available in surgical pathology. Notwithstanding controversy regarding its nomenclature, malignant fibrous histiocytoma involving the penis is exceedingly rare, with only 4 cases previously described, to our knowledge. CASE: An uncircumcised 73-year-old male presented with a painless, granular, partially necrotic lesion beneath the penile foreskin. There was no history of sexually transmitted disease, constitutional symptoms or dysuria. Examination of penile shaft, testicles, spermatic cord and inguinal lymph nodes were unremarkable. Biopsy revealed a markedly pleomorphic sarcoma. Subsequent, partial penectomy revealed the same lesion with an adjacent area of squamous cell carcinoma in situ. CONCLUSION: Malignant fibrous histiocytoma remains a diagnosis of exclusion. The investigation requires extensive tumor sampling in search of areas of differentiation and a complete battery of immunohistochemical markers. Therapeutically important entities in the differential diagnosis that must be ruled out include other poorly differentiated sarcomas, sarcomatoid squamous cell carcinoma and desmoplastic melanoma.     

An unusual pleomorphic sarcoma in a hybrid mallard

An unusual pleomorphic sarcoma from a hybrid mallard (Anas platyrhynchos) is described. Rhabdomyosarcoma was considered in the original differential diagnoses but rejected due to lack of specific characteristics generally seen in these tumors. The histologic characteristics described are consistent with mammalian sarcomas recorded in the literature as malignant fibrous histiocytoma.     

Establishment and characterization of a human malignant fibrous histiocytoma cell line (K-MFH-1)]

We established and characterized a cell line (K-MFH-1) of human malignant fibrous histiocytoma in vitro and in nude mice. The cells have been kept in culture for nearly 4 years in 121 subcultures in vitro and transferred serially in nude mice in 10 generations. The original tumor histology was epithelioid, angiomatoid variant of MFH, but cells in culture showed a variety of morphology. Chromosomal study disclosed that this tumor was monoclonal in origin. Electron microscopic study revealed that cells had some epithelial characteristics as well as histiofibroblastic features. Tumor cell morphology gradually shifted from epithelioid to myxomatous fibroblastic forms after serial transplantations in nude mice. Some tumors showed an extensive vascular proliferation like angioma suggesting that tumor cells have released angiogenic factors.     

High serum alkaline phosphatase cooperating with MMP-9 predicts metastasis and poor prognosis in patients with primary osteosarcoma in Southern China

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.     

Fine needle aspiration and touch imprint cytology of a malignant fibrous histiocytoma of the spermatic cord. Case report

BACKGROUND: No cytologic reports on spermatic cord sarcomas have been published. CASE: A 64-year-old man presented with a slowly growing, painless, left spermatic cord enlargement. Fine needle aspiration (FNA) obtained < 1 mL of bloody fluid consisting of solitary, mark-edly anaplastic and pleomorphic tumor giant cells occasionally arranged in small fragments. Rare atypical spindle cells could be observed. Some reactive lymphocytes were observed intermingled with tumor cells. Immunohistochemistry displayed vimentin reactivity and negativity for keratins and leukocytic common antigen. The specimen removed showed a well-circumscribed, 30-mm, yellowish solid tumor. Touch imprints displayed pleomorphic tumor cells showing intense anisonucleosis; a moderate amount of clear, sometimes microvacuolated cytoplasm; and tissue fragments with a storiform pattern. Histologic examination revealed microscopic and immunohistochemical features of malignant fibrous histiocytoma (MFH) arising in soft tissues of the spermatic cord. CONCLUSION: FNA of a spermatic cord lesion may reveal a pleomorphic sarcoma. A pleomorphic appearance together with some spindle elements and compatible immunocytochemistry could help diagnose spermatic cord MFH. This is one of the few reports dealing with FNA cytology of paratesticular tumors and the first report, to the best of our knowledge, showing the cytologic characteristics of a case of spermatic cord MFH.     

Primary malignant fibrous histiocytoma of the lung. Fine needle aspiration cytologic features

A case of primary malignant fibrous histiocytoma of the lung occurring in a 71-year-old woman is presented. The preoperative aspiration cytology showed a large-cell, undifferentiated, malignant neoplasm suggestive of carcinoma. Subsequent histologic examination revealed a primary malignant fibrous histiocytoma. The diagnosis was confirmed by electron microscopic and immunohistochemical studies. Cytologic features of this rare primary pulmonary sarcoma are discussed.     

Neutrophilic phagocytosis by tumor cells. A feature to consider in the differential diagnosis in fine needle aspiration smears

OBJECTIVE: To analyze neutrophilic phagocytosis by tumor cells in fine needle aspirate (FNA) smears from different types of tumor. STUDY DESIGN: A retrospective review of a total of 7 cases showing prominent neutrophilic phagocytosis by tumor cells in FNA smears during the period July 2003-December 2004. RESULTS: This feature was seen in malignant fibrous histiocytoma and poorly differentiated renal cell carcinoma in addition to giant cell carcinoma of the lung. CONCLUSION: Neutrophilic phagocytosis by tumor cells is seen in FNA smears and on cytomorphology. The differential diagnoses should include both pleomorphic sarcomas and carcinomas.     

Leiomyosarcomas: three cases with desmin positive tumour cells, lacking ultrastructural features of smooth muscle cells

A combined study of light and electron microscopy and of immunolabelling of three pleomorphic spindle cell sarcomas is presented. The light and electron microscopic features of these sarcomas were most compatible with those described for malignant fibrous histiocytoma (MFH, pleomorphic-storiform subtype). Electronmicroscopically undifferentiated and fibroblast-like cells, fibrohistiocytes and multinucleated histiocytes were observed. Characteristics belonging to smooth muscle cells were absent. By immunostaining, vimentin and desmin could be observed in tumour cells of all three cases, at least on frozen sections. Other markers such as alpha 1-antichymotrypsin, S-100 proteins, laminin, collagen IV and markers specific for skeletal muscle cells (myoglobin, actin and myosin specific for skeletal muscle) could not be demonstrated. These findings indicate that three MFH's are, in fact, poorly differentiated variants of smooth muscle tumours. It is concluded that immunophenotyping is very useful for this type of neoplasm.     

Evaluation of P-glycoprotein expression in soft tissue sarcomas of the extremities

Soft tissue sarcomas comprise a heterogeneous group of mesenchymal tumors accounting for less than one-percent of adult neoplasms. In the last few years, the use of adjuvant chemotorapy has been proposed for the treatment of these lesions in order to obain a better systemic control, but its usefulness is still controversial. In this study, we evaluated whether P-glycoprotein, a membrane protein strictly associated with multidrug resistance, is overexpressed in soft tissue sarcomas. By using human multidrug resistant sarcoma cell lines as controls, we analyzed P-glycoprotein expression in 34 primary and in 23 relapsed soft tissue sarcomas of the extremities. Overexpression of P-glycoprotein was found in 6 out of 34 primaries (18%) and in 8 out of 23 relapses (35%). In particular, in malignant fibrous histiocytoma, the most frequent soft tissue sarcoma of adults, P-glycoprotein overexpression was found in 23% of primary untreated cases, in agreement with the reported relapse rate of this tumor after surgery and chemotherapy. These data suggest that, in soft tissue sarcomas, overexpression of P-glycoprotein may be of prognostic value and that the assessment of P-glycoprotein expression may be useful for the design of chemotherapy protocols.Abbreviations MDR multidrug-resistance - STS soft tissue sarcomas     

Analysis of Multiple Sarcoma Expression Datasets: Implications for Classification,Oncogenic Pathway Activation and Chemotherapy Resistance

Background

Diagnosis of soft tissue sarcomas (STS) is challenging. Many remain unclassified (not-otherwise-specified, NOS) or grouped in controversial categories such as malignant fibrous histiocytoma (MFH), with unclear therapeutic value. We analyzed several independent microarray datasets, to identify a predictor, use it to classify unclassifiable sarcomas, and assess oncogenic pathway activation and chemotherapy response.

Methodology/Principal Findings

We analyzed 5 independent datasets (325 tumor arrays). We developed and validated a predictor, which was used to reclassify MFH and NOS sarcomas. The molecular “match” between MFH and their predicted subtypes was assessed using genome-wide hierarchical clustering and Subclass-Mapping. Findings were validated in 15 paraffin samples profiled on the DASL platform. Bayesian models of oncogenic pathway activation and chemotherapy response were applied to individual STS samples. A 170-gene predictor was developed and independently validated (80-85% accuracy in all datasets). Most MFH and NOS tumors were reclassified as leiomyosarcomas, liposarcomas and fibrosarcomas. “Molecular match” between MFH and their predicted STS subtypes was confirmed both within and across datasets. This classification revealed previously unrecognized tissue differentiation lines (adipocyte, fibroblastic, smooth-muscle) and was reproduced in paraffin specimens. Different sarcoma subtypes demonstrated distinct oncogenic pathway activation patterns, and reclassified MFH tumors shared oncogenic pathway activation patterns with their predicted subtypes. These patterns were associated with predicted resistance to chemotherapeutic agents commonly used in sarcomas.

Conclusions/Significance

STS profiling can aid in diagnosis through a predictor tracking distinct tissue differentiation in unclassified tumors, and in therapeutic management via oncogenic pathway activation and chemotherapy response assessment.     

Malignant fibrous histiocytoma of the chest wall masquerading as medullary breast carcinoma: a case report

BACKGROUND: Cytologic diagnosis of malignant fibrous histiocytoma can be problematic, as these neoplasms are known to mimic multiple other conditions. CASE: A fine needle aspirate from a 60-year-old woman was diagnosed at 2 institutions as medullary carcinoma of the breast. The patient received neo-adjuvant chemoradiotherapy before the tumor war excised. Gross pathologic examination and histomorphology on routine staining were compatible with the cytologic diagnosis. The accurate diagnosis of pleomorphic-storiform-type malignant fibrous histiocytoma was a surprise and was established with immunocytochemical stains. In retrospect, it was thought that clinical and radiologic overlap, creating a high index of suspicion for a breast neoplasm and compounding the cytologic appearance of a medullary carcinoma with spindle cell metaplasia and syncytial cells, was responsible for the error. CONCLUSION: This case highlights a potential cytodiagnostic pitfall and the importance of establishing a definitive tissue diagnosis in the face of equivocal cytologic findings.     

Malignant fibrous histiocytoma in a baboon

A recurrent, invasive, soft tissue tumor was observed in the subcutaneous tissue of the back of an adult baboon (Papio cynocephalus). The neoplasm was diagnosed as a malignant fibrous histiocytoma.     

Malignant fibrous histiocytoma of the esophagus. Report of a case with cytologic, immunohistologic and ultrastructural studies.

A 65-year-old man presented with an esophageal polyp that proved to be malignant pleomorphic fibrous histiocytoma, extremely rare at that location. Diagnosis of the abrasion cytology specimen was confirmed by immunohistochemical and electron microscopic methods. Differential diagnostic problems of pleomorphic soft tissue tumors and epithelial tumors mimicking them are discussed.     

Cytologic features of a primary myxoid malignant fibrous histiocytoma arising in the uterus: a case report.

BACKGROUND: Primary malignant fibrous histiocytoma (MFH) of the uterus is extremely rare. The 10 cases reported in the literature all involved the pleomorphic variant, and to the best of our knowledge, the myxoid variant has not been reported before. We describe the cytologic findings of primary uterine myxoid MFH in relation to the myxoid component, potentially leading to an incorrect diagnosis. CASE: A 68-year-old woman presented with a primary uterine tumor. Endometrial cytology showed numerous loosely arranged, spindle-shaped fibroblastlike cells; atypical histiocytelike cells; and giant cells with a necrotic background. The overall cytologic picture was of a degenerated pleomorphic leiomyosarcoma with an inconclusive diagnosis. A diagnosis of myxoid MFH was established after electron microscopic and immunohistochemical studies of the primary tumor and tumor transplanted, as primary cultured cells, in nude mice. The patient underwent an exploratory laparotomy and died of tumor progression 38 days after the initial consultation, without treatment. CONCLUSION: Because of overlapping cytologic features among uterine sarcomas with myxoid stroma, it is important to recognize the histiocytic lineage of tumor cells by immunohistochemistry and electron microscopy in various presentations of fresh samples.     

Fine needle aspiration cytology of sarcomas metastatic to the lung

A review was made of the morphologic features of cells aspirated from 17 sarcomas (5 malignant fibrous histiocytomas, 3 fibrosarcomas, 3 leiomyosarcomas, 3 endometrial stromal sarcomas, 1 osteosarcoma and 2 poorly differentiated sarcomas) metastatic to the lung, paying particular attention to the morphologic differences between the cells of sarcoma and carcinoma and between the cells of the different types of sarcoma. In general, sarcomas were characterized by loosely cohesive, rather flat, cellular aggregates and isolated cells. Three-dimensional cell balls or clusters were not present in any case. Cellular pleomorphism was a common, though not invariable, feature. Each type of sarcoma showed some morphologic distinctiveness; however, certain morphologic features were common to more than one type of sarcoma. By comparing the cytologic features of metastatic sarcomas in aspirates with the histologic features of the primary neoplasms, it should usually be possible to decide if a pulmonary lesion is a metastatic sarcoma.     

Primary hepatic malignant fibrous histiocytoma: a case report and review of the literature

Primary malignant fibrous histiocytoma (MFH) of the liver remains extremely rare with only several cases having been reported in literature. We report a case of hepatic MFH in a 53-year-old man who presented with upper abdominal pain, and weight loss for one month. Ultrasound and computed tomography (CT) scan showed a large mass with fine tumor vessels over the left lobe of the liver. Histopathological findings indicated a mesenchymal tumor consisting of spindle cells in storiform pattern intermingled with histiocyte-like cells and giant cells. Immunohistochemically, most tumor cells expressed vimentin, alpha-1 anti-chymotrypsin, alpha-1 antitrypsin and CD68. Morphological and immunohistochemical findings support that the tumor should be classified as a primary malignant fibrous histiocytoma. The literatures is briefly reviewed.     

Reduced expression of INK4a/ARF genes in stem-like sphere cells from rat sarcomas

The presence of cancer stem cells, in both hematopoietic and solid malignancies, has been recently linked to their pathogenesis. We aimed to identify the characteristics and stem-like properties of sphere-colony forming cells in rat osteosarcoma and malignant fibrous histiocytoma cell lines. The results showed that both cell lines possessed an ability to form spherical, clonally expanding colonies in anchorage-independent, serum-starved conditions in N2/1% methylcellulose medium. The sphere cells showed stem-like properties with the ability to self-renew, and expressed the stem cell-related STAT3 and Bmi1 genes. Interestingly, spheres from both sarcomas remarkably decreased the expression of INK4a/ARF locus genes, p16(INK4a) and p19(ARF), which could be related to the resistance against cell senescence and apoptosis. Spheres showed strong tumorigenicity with metastatic potential in vivo via the inoculation into syngeneic rats, suggesting the presence of these populations might contribute to the tumor development such as metastasis via the resistance to apoptotic stimuli.     

Eosinophilic globule cells in mouse MFH-like sarcomas

Light and electron microscopic observations were made on eosinophilic globule (EG) cells found in 27 subcutaneous malignant fibrous histiocytoma (MFH)-like sarcomas in aged ICR mice. These tumors, which were composed of fibroblast-like cells as the major component, with small numbers of histiocyte-like cells and undifferentiated cells, showed one or more of five growth patterns: storiform, pleomorphic, fascicular, myxoid, and hemangiopericytoma-like. EG cells were interspersed among the tumor cells and were also present in metastatic lesions. They were pleomorphic in shape and contained various numbers of cytoplasmic globules, which were positive with the periodic acid-Schiff reaction and resistant to diastase digestion. Electron microscopic observation revealed that these cells had small finger-like and pseudopodia-like projections, and contained varied numbers of characteristic osmiophilic globules and glycogen particles in the cytoplasm which seemed to become more abundant as the cells differentiated. The osmiophilic globules consisted of a dense homogeneous core and a marginal area rich in small vesicular membranous structures. A small population of EG cells exhibited features suggesting differentiation to fibroblasts; these were characterized by an increased amount of rough endoplasmic reticulum. It is suggested that the EG cell is a neoplastic cell, perhaps derived from a primitive mesenchymal cell, although an inflammatory cell origin is also possible.     

Eosinophilic globule cells in mouse MFH-like sarcomas. Light and electron microscopic studies

Light and electron microscopic observations were made on eosinophilic globule (EG) cells found in 27 subcutaneous malignant fibrous histiocytoma (MFH)-like sarcomas in aged ICR mice. These tumors, which were composed of fibroblast-like cells as the major component, with small numbers of histiocyte-like cells and undifferentiated cells, showed one or more of five growth patterns: storiform, pleomorphic, fascicular, myxoid, and hemangiopericytoma-like. EG cells were interspersed among the tumor cells and were also present in metastatic lesions. They were pleomorphic in shape and contained various numbers of cytoplasmic globules, which were positive with the periodic acid-Schiff reaction and resistant to diastase digestion. Electron microscopic observation revealed that these cells had small finger-like and pseudopodia-like projections, and contained varied numbers of characteristic osmiophilic globules and glycogen particles in the cytoplasm which seemed to become more abundant as the cells differentiated. The osmiophilic globules consisted of a dense homogeneous core and a marginal area rich in small vesicular membranous structures. A small population of EG cells exhibited features suggesting differentiation to fibroblasts; these were characterized by an increased amount of rough endoplasmic reticulum. It is suggested that the EG cell is a neoplastic cell, perhaps derived from a primitive mesenchymal cell, although an inflammatory cell origin is also possible.