Cutaneous malignant fibrous histiocytoma. A rare but serious malignancy

We report 3 cases of malignant fibrous histiocytoma occurring as primary neoplasms of the skin. The first case developed in a leg ulcer of traumatic origin. The second developed on the lower lip at the site of a squamous cell carcinoma which had been treated by radiotherapy. The third arose on a calf at a site of previous surgery. The literature on this malignancy is reviewed, with emphasis on cutaneous involvement.     

A case of malignant fibrous histiocytoma

A case of malignant fibrous histiocytoma is reported. Due to the histological features of cellular pleomorphism and myxoid degeneration seen also in other soft tissue sarcomas (rhabdomyosarcoma, liposarcoma), differential diagnosis was difficult. However, electron microscopic studies led to a distinct diagnosis of malignant fibrous histiocytoma.     

Aneurysmal Benign Fibrous Histiocytoma with Atrophic Features

Aneurysmal benign fibrous histiocytoma is an uncommon pathologic variant of dermatofibroma. In addition to the features of a typical dermatofibroma, it has large cleft-like or cavernous blood-filled spaces with numerous hemosiderin pigments. It should be differentiated from angiomatoid malignant fibrous histiocytoma, malignant melanoma, and vascular tumors such as Kaposi''s sarcoma and angiosarcoma. Atrophic dermatofibroma is also a rare variant of dermatofibroma, and the combination of aneurysmal and atrophic features is rarer still. We report a case of aneurysmal benign fibrous histiocytoma with atrophic features in a 27-year-old male who had a grayish-brown atrophic patchy lesion on his back for 2 years.     

Malignant fibrous histiocytoma of the scalp. Multidisciplinary treatment

BACKGROUND: Malignant fibrous histiocytomas (MFH) are uncommon in the skin, and even less frequent on the scalp. On the scalp they are often very difficult to excise and it is even more difficult to close the resulting wound. OBJECTIVE: To review all malignant fibrous histiocytomas diagnosed and treated in our Department during the past 6 years, and to describe the multidisciplinary procedure employed to treat one special case of aggressive malignant fibrous histiocytoma on the scalp that recurred twice. RESULT: Malignant fibrous histiocytomas represent 0.01% of malignant cutaneous tumors in our area. The immediate results after a multidisciplinary treatment performed on a recurrent malignant fibrous histiocytoma located on the scalp were excellent, but recurrence was observed 6 months later. Two years later we have also treated another case of MFH on the scalp. The same surgical technique was performed, but the patient received high-dose-methotrexate-based neoadjuvant chemotherapy (HD-MTX). One year later, this patient is still alive and no signs of recurrence have been detected. CONCLUSION: When malignant fibrous histiocytoma occurs on the scalp it must be treated immediately by means of an excision with a large peripheral edge of 2 cm from the visual or CAT limits of the lesion, since the first treatment must be definitive.     

Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update

The fibrohistiocytic tumors of intermediate malignancy are uncommon mesenchymal tumors, which typically occur in the skin and subcutis and which may pose significant problems for the dermatopathologist. This article reviews the clinical, histopathologic, and genetic features of dermatofibrosarcoma protuberans, giant cell fibroblastoma, angiomatoid (malignant) fibrous histiocytoma, plexiform fibrous histiocytoma, and soft tissue giant cell tumor (of low malignant potential). The differential diagnosis of these tumors with a variety of benign and fully malignant cutaneous soft tissue neoplasms is discussed.     

A Case of Subcutaneous Malignant Fibrous Histiocytoma Circumscribed by Fibrous Tissue

We report a case of malignant fibrous histiocytoma (MFH) located in the subcutaneous tissue on the right axilla. We excised the tumor sufficiently beyond the clinical margin. It was pathologically diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma almost completely circumscribed by fibrous tissue, including fascicles of fibroblasts; this is a rare histological picture. The tumor has not recurred for three years. Although MFH frequently undergoes metastasis, the circumscribed-type subcutaneous MFH characteristic of superficiality and of histologically well-defined structure seems to have a relatively more favorable prognosis after adequate radical excision.     

Fibrohistiocytic skin tumors

The fibrohistiocytic tumors of the skin are a heterogeneous group of dermal/subcutaneous mesenchymal neoplasms which show fibroblastic, myofibroblastic and histiocytic (macrophage‐like) differentiation, often one beside the other in the same tumor. “Fibrohistiocytic” means in this context the morphologic similarity of the cells with fibroblasts and histiocytes. The WHO classification of 2005 includes the following entities as fibrohistiocytic tumors of the skin: Benign: 1. Fibrous histiocytoma (FH)/(synonymous: Dermatofibroma. Variants of FH: 1a. cellular fibrous histiocytoma, 1b. atypical (pseudosarcomatous) fibrous histiocytoma, 1c. aneurysmatic fibrous histiocytoma, 1d. epithelioid fibrous histiocytoma; 2. dermatomyofibroma; 3. (juvenile) xanthogranuloma. Intermediate: 4. plexiform fibrohistiocytic tumor; 5. dermatofibrosarcoma protuberans; 6. atypical Fibroxanthoma. Malignant: 7. malignant fibrous histiocytoma. All these entities are reviewed in this paper with particular attention devoted to differential diagnostic considerations.     

Primary Malignant Fibrous Histiocytoma of Skin

Three fibrous histiocytomas, primary in skin, were studied by light and electron microscopy and by frozen section histochemistry. The term malignant was applied to cutaneous tumors which demonstrated aggressive multinodular local growth, including angioinvasion and/or extension into bone, muscle, and fascia. Metastases were not found. Strongly positive reactions for hydrolytic enzymes, particularly acid phosphatase will help differentiate malignant fibrous histiocytoma from most other primary soft tissue malignancies of skin. Electron microscopic studies reaffirm the presence of both histiocyte-like and fibroblast-like cells. Langerhans' granules were not identified. Primary malignant fibrous histiocytoma of skin may have a prognosis superior to homologous tumors arising in deeper soft tissue and the retroperitoneum.     

Fibroblastic/myofibroblastic sarcoma of the skin: a report of five cases

BACKGROUND: A number of malignant soft tissue tumors, particularly those of fibroblastic and fibrohistiocytic derivation, have been found to display myofibroblastic differentiation focally. The term myofibroblastic sarcoma, a controversial presumably distinctive entity, defines a malignant soft tissue tumor in which myofibroblasts are quantitatively the predominant cell type. METHODS: Five cases of cutaneous spindle-cell sarcomas showing fibroblastic-myofibroblastic differentiation with predominance of fibroblasts were retrieved from the files of three large centers of dermatopathology. Tumors were analyzed histopathologically, immunophenotypically, and, in two cases, ultrastructurally. Results were compared with those previously reported in fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma. RESULTS: Immunophenotypic and ultrastructural profiles of the cases analyzed in this series were closer to fibrosarcoma and to malignant fibrous histiocytoma than to myofibroblastic sarcoma by virtue of quantitative predominance of fibroblasts over myofibroblasts. On the other hand, histopathologic findings were in keeping with those reported in myofibroblastic sarcoma. CONCLUSIONS: Our series highlights the intrinsic problems in attaching certain cutaneous sarcomas with fibroblastic-myofibroblastic differentiation to one of the recognized entities and gives support to the hypothesis that fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma are related histogenetically.     

Benign cellular fibrous histiocytoma with erosion of the phalanx

Fibrohistiocytic tumors are characterized by the presence of fibroblast like spindle cells and histiocytes. The benign fibrous histiocytoma (dermatofibroma, BFH) as well as the malignant dermatofibrosarcoma protuberans (DFSP) and the malignant fibrous histiocytoma (MFH) belong to this group. A recurrent painful, hard 2 cm tumor on the left hallux of a 54-year-old woman led to an erosion of the underlying phalanx. The patient had suffered from ingrown toenails for more than 10 years. Histologically there was a deep penetrating fibrohistiocytic tumor that grew in a storiform pattern with interspersed foam cells. The tumor was CD34 negative and mitoses were scarce. The diagnosis was benign cellular fibrous histiocytoma (BZFH). BZFH belong to the group of BFH with a high recurrence rate especially after incomplete removal. Damage to the underlying bone has not been reported so far.     

Pedunculated hemangiopericytoma-like tumor: peculiar fibroepithelial polyp or fibrous histiocytoma variant

Abstract:  Pathologists are continually challenged with the difficult task of discriminating between innocuous disease processes and potentially malignant entities. Apropos of this concern, we present a series of three cutaneous polypoid lesions that simulated fibroepithelial polyp, yet upon close scrutiny yielded histologic features of solitary fibrous tumor (SFT) or hemangiopericytoma. These pedunculated lesions showed a storiform pattern of spindled cells with interspersed gaping vascular channels reminiscent of SFT or hemangiopericytoma. Interestingly, the immunohistochemical staining profile of these lesions was negative for CD34 and positive for bcl-2 and factor XIIIa. These findings were discordant with SFT and suggest a relationship with fibrous histiocytoma. We propose that this entity represents a hitherto described variant of fibrous histiocytoma known as pedunculated hemangiopericytoma-like fibrous histiocytoma.     

Cancer family syndrome associated with multiple malignant melanomas and a malignant fibrous histiocytoma

A 64-year-old female with the cancer family syndrome suffered from multiple malignant melanomas and a malignant fibrous histiocytoma. She has shown unusually long survival which is a feature of these patients.     

Postirradiation malignant fibrous histiocytoma. A case report with a review of postirradiation sarcoma in the Japanese literature

A fatal case of postirradiation malignant fibrous histiocytoma was reported, with a review of postirradiation sarcoma in the Japanese literature.     

Malignant fibrous histiocytoma of the skin with marked inflammatory infiltrate: a sarcoma mimicking malignant lymphoma

Three cases of malignant fibrous histiocytoma of the skin with a marked inflammatory infiltrate in the stroma are reported. The inflammatory infiltrate, composed mainly of T-lymphocytes, obscured the nature of the neoplasms, and immunohistochemical studies were required to establish the diagnosis. Two tumors arose in the sun-damaged skin of the face, and one tumor arose in the chest wall. One patient developed a local recurrence with histopathologic findings similar to those observed in the original lesion, including the inflammatory infiltrate. Possible differential diagnoses include large cell lymphoma, inflammatory pseudotumor, inflammatory leiomyosarcoma, and spindle cell squamous cell carcinoma. The presumed rarity of inflammatory changes in malignant fibrous histiocytoma of the skin is supported by the absence of reported cases.     

恶性纤维组织细胞瘤1例

报告1例恶性纤维组织细胞瘤。患者男,78岁,左胫前色素性结节10余年,近两年结节缓慢增大呈紫黑色斑块,无明显自觉症状。组织病理检查示,肿瘤中成分呈多形性表现,主要由增生的内皮细胞构成,部分细胞似成纤维细胞样交织排列呈游涡状,有些细胞似组织细胞样,可见形状怪异的多核巨细胞。     

Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma

Neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.     

Malignant fibrous histiocytoma arising from discoid lupus erythematosus

While the association of discoid lupus erythematosus (DLE) and subsequent squamous cell carcinoma is well known, there are no reports in the English-language literature concerning the association of DLE and soft-tissue sarcoma. We describe a patient with DLE and malignant fibrous histiocytoma.     

Malignant fibrous histiocytoma and atypical fibroxanthoma in renal transplant recipients

BACKGROUND: Allograft recipients are at increased risk for skin cancer. The incidence of cutaneous squamous cell carcinoma is 50-250 times higher than in the age-matched control population, and basal cell carcinoma is about 10 times more frequent. The incidence of Kaposi's sarcoma is increased 400 to 500 times over that in a control population of the same ethnic origin. However, the incidence of other types of cutaneous sarcoma in organ allograft recipients is largely unknown. CLINICAL OBSERVATION: Within a 2-year-period, we observed 2 patients with cutaneous malignant fibrous histiocytoma and 1 patient with atypical fibroxanthoma among a cohort of 642 renal transplant recipients. For comparison, the incidence for dermatofibrosarcoma protuberans which is the commonest type of cutaneous sarcoma, is 0.45/100,000 persons/year in the non-immunocompromised population. Our observation represents an incidence of 156/100,000/ year (95% confidence interval Cl 28-489/100,000/year) for cutaneous malignant fibrous histiocytoma and of 78/100,000/year (95% CI 4-368/ 100,000/year) for atypical fibroxanthoma. CONCLUSION: To our knowledge, this is the first report on an elevated incidence of cutaneous malignant fibrous histiocytoma and of atypical fibroxanthoma in renal transplant recipients. Future cohort studies on malignancies in organ allograft recipients should aim at defining this risk more exactly.     

Myofibroblasts in malignant fibrous histiocytoma-histochemical, immunohistochemical, ultrastructural and tissue culture studies

Spindle cells found in malignant fibrous histiocytoma (MFH) were examined using histochemical, immunohistochemical, ultrastructural and tissue culture studies. They were deeply eosinophilic on haematoxylin-eosin staining, strongly PAS-positive and darkly stained with nicotinamide adenine dinucleotide tetrazolium reductase and menadione linked 2-glycerophosphate dehydroge-nase. They also labelled with anti-S-100 and actin antibodies. Fuchsin positive intra-cytoplasmic filaments were recognizable with phosphotungstic acid haematox-ylin stains. Ultrastructurally, the spindle cells had an indented nucleus and elongated cytoplasm containing myofilaments with electron dense bodies. In tissue culture, the spindle cells had plump cytoplasm and were closely apposed to each other within a sheath of fibro-blasts. Using electron microscopy the cultured spindle cells were found to contain numerous glycogen particles, rod-shaped mitochondria, rough endoplasmic reticulum and bundles of myofilaments within the cytoplasm. The spindle cells were identified as ‘myofibroblasts’ and the presence of myofibroblasts in MFH was thus confirmed. The function of myofibroblasts in malignant fibrous histiocytoma is discussed.     

Malignant fibrous histiocytoma

A 74-year-old man presented with a malignant fibrous histiocytoma (MFH) on the lower leg. Wide local excision was performed and no recurrence has occurred to date. The pathological features which distinguish MFH from other sarcomas are now well denned and deserve wider recognition.