Benign fibrous histiocytoma of the eyelid with an unusual clinical presentation

Benign fibrous histiocytoma is a common soft tissue tumor that can be deep or superficially located. Although the deep type of fibrous histiocytoma has a predilection for the orbit, the eyelids are an unusual location for the cutaneous type. A 42-year-old woman had bilateral yellowish nodular masses of the eyelids for two years. Pathological examination after excision revealed benign fibrous histiocytoma. Our case is an unusual clinical presentation of cutaneous fibrous histiocytoma as well as a rare location such as the eyelids.     

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.     

Aneurysmal fibrous histiocytoma: an unusual variant of cutaneous fibrous histiocytoma

Aneurysmal fibrous histiocytoma (AFH) (Santa-Cruz DJ, Kyriakos M. Aneurysmal ('Angiomatoid') fibrous histiocytoma of the skin. Cancer 1981;47:2053-2061) is a distinct but poorly recognized clinicopathological variant of cutaneous fibrous histiocytoma (CFH) that may result from the slow extravasation of blood into the tumour. The resulting lesion can have a very different clinicopathological appearance resulting in diagnostic confusion. We describe a patient with an AFH that presented as a pigmented nodule on the foot and discuss clinical recognition and histological differentiation from other tumours.     

Palisading cutaneous fibrous histiocytoma

We report 6 cases of an unusual variant of cutaneous fibrous histiocytoma in which nuclear palisading is a prominent feature. The lesions were equally distributed between males and females with a widely variable age range. Half of the cases occurred on the digits. Histopathologically, the lesions were characterized by areas of nuclear palisading with formation of Verocay-like bodies in addition to the more typical features of the "fibrous" variant of cutaneous fibrous histiocytoma. The differential diagnostic features between these lesions and those of other tumors in which nuclear palisading is seen are discussed.     

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.     

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.     

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.     

Metastatic aneurysmal fibrous histiocytoma in a 20-year-old woman: A rare case report with review of the literature and discussion of its genomic features

Aneurysmal fibrous histiocytoma is an uncommon variant of cutaneous fibrous histiocytomas with a local recurrence rate of 19%. We present a case of aneurysmal fibrous histiocytoma in a 20-year-old female with a regional lymph node metastasis and subsequent satellite nodule. The patient initially presented with a 1-month history of two palpable nodules in left lower anterior shoulder and left axilla. Needle core biopsies from both lesions revealed an atypical spindle cell neoplasm with a differential diagnosis of aneurysmal fibrous histiocytoma and angiomatoid fibrous histiocytoma. The axillary dissection confirmed a metastatic deposit in 1 out of 22 lymph nodes. At 6 months a satellite nodule arose between the resection scar and the axilla histopathologically demonstrating a cellular spindle cell nodule at the dermis subcutaneous junction with large, blood-filled pseudovascular spaces lined by histiocytes. The periphery of the lesion showed collagen trapping without a lymphoplasmacytic infiltrate. The lesional cells were diffusely positive for CD10 and focally for CD68 and Illumina RNA fusion panel sequencing was negative. Herein we present this case of metastatic aneurysmal fibrous histiocytoma with review of the literature and discussion of biology, cytogenetic alterations, and differential diagnosis.     

The value of immunohistochemistry in atypical cutaneous fibrous histiocytoma

Atypical cutaneous fibrous histiocytoma is a rare variant of dermatofibroma/fibrous histiocytoma characterized by striking atypia, thus resembling atypical fibroxanthoma. We studied 9 examples of ACFH histopathologically and immunohistochemically to investigate the nature of these atypical cells. Histology revealed ill-defined skin nodules, which were polypoid in 6 cases. A minority of mononuclear and giant cells (< 5%) revealed striking pleomorphism and showed large nuclei with prominent nucleoli. Immunohistologically, the atypical cells expressed vimentin, but were negative for S-100 protein, the keratin marker MNF116, alpha smooth muscle actin, CD34, factor XIIIa, and monocyte/macrophage markers Ki-M1p, KP1 (CD68), and MAC387. Positivity for MiB1 was very modest (< 1%) and limited to small- and medium-sized, inconspicuous cells. Multinucleate giant cells proved to be heterogenous, on one hand cells with differentiation toward macrophages with positivity for Ki-M1p and KP1, on the other toward fibroblasts positive for vimentin only. These immuno-histochemical results for differentiation markers in atypical cutaneous fibrous histiocytoma are similar to our previous findings and data in atypical fibroxanthoma; MiB1 helps to separate these entities from each other as the latter shows a very high proportion of proliferative atypical cells corresponding to the numerous mitoses seen in routine sections.     

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.     

Postirradiation malignant fibrous histiocytoma

A slowly growing, firm, asymptomatic tumor developed in the skin overlying the left scapula of a 72-year-old woman during a six-month period. Twenty-seven years earlier, she had radiation treatment to that site for an intraductal carcinoma of the left breast. The histopathologic diagnosis was malignant fibrous histiocytoma (MFH). The rarity of this late complication of radiation therapy is reflected by a few reports in the medical literature of cutaneous MFH as late radiation sequelae.     

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.     

Cutaneous Metastatic Undifferentiated Pleomorphic Sarcoma from a Mediastinal Sarcoma

Undifferentiated pleomorphic sarcoma, known as malignant fibrous histiocytoma, is a malignant neoplasm that arises in both soft tissue and bones. In 2002, the World Health Organization declassified malignant fibrous histocytoma as a formal diagnostic entity and renamed it ''undifferentiated pleomorphic sarcoma not otherwise specified.'' It most commonly occurs in the lower extremities and rarely metastasizes cutaneously. We report a case of cutaneous metastatic undifferentiated pleomorphic sarcoma of the buttocks occurring in a 73-year-old man diagnosed with mediastinal sarcoma 4 years previously. He first noticed the mass approximately 2 months previously. Histological findings with immunomarkers led to a final diagnosis of cutaneous metastatic sarcoma from mediastinal undifferentiated pleomorphic sarcoma.     

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.     

Polypoid dermatofibroma with a slim pedicle: a case report

Dermatofibroma, also called cutaneous fibrous histiocytoma or sclerosing hemangioma, is a fairly common, benign cutaneous tumor. Polypoid dermatofibroma is an unusual and poorly recognized form of this entity. We describe a peculiar case of this variant presenting with a unique morphology characterized by a round flat shape with a slim pedicle, although its histopathological picture was rather typical of that of ordinary dermatofibroma.     

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.     

A 10‐year analysis of cutaneous mesenchymal tumors (sarcomas and related entities) in a skin cancer center

Background Mesenchymal neoplasms (sarcomas) of skin are rare. Patients with sarcomas were analyzed over the last decade. Methods Over a 10‐year period, we conducted a retrospective analysis of patients diagnosed and treated in an urban academic teaching hospital in Saxony, Germany. Clinical and pathologic files were used. Results We identified 65 adult patients with 67 primary cutaneous sarcomas. The mean age was 73.1 (±15.5) years with a male predominance (78.5%). None of the sarcomas was detected by a skin cancer screening program. The diagnosis was atypical fibroxanthoma (n = 41 patients with 43 tumors), cutaneous angiosarcoma (eight), dermatofibrosarcoma protuberans (two), nodular epithelioid cell sarcoma (one), Kaposi sarcoma (three), leiomyosarcoma (five), malignant fibrous histiocytoma (two), fibromyxoid sarcoma (one), and cutaneous angiomyxoma (two). The preferred tumor localization was the head and neck area (44 patients). Follow‐up was 0.5–5.5 years (mean 18 ± 12 months). We observed metastatic spread of atypical fibroxanthoma in 12.5%, demonstrating that this type of sarcoma can run an aggressive course. Mohs surgery is still the cornerstone of treatment, although new options in palliative or adjuvant treatment are available. Conclusions Mesenchymal neoplasms (sarcomas) are an important group of cutaneous malignancies. Awareness needs to be improved.     

Dermatofibroma with osteoclast-like giant cells.

Dermatofibroma (DF), or cutaneous fibrous histiocytoma, is a common cutaneous tumor with many variants that may arise from alterations in the morphology and composition of its various components. One type that has not received much attention is DF with osteoclast-like giant cells. Two cases of this rare tumor are described. The importance of this tumor lies in the possible histologic confusion with a variety of benign and malignant neoplasms, including giant cell tumor of tendon sheath, giant cell tumor of bone, and giant cell reparative granuloma.     

Benign fibrous histiocytoma (dermatofibroma) of the face: clinicopathologic and immunohistochemical study of 34 cases associated with an aggressive clinical course.

Thirty-four cases of fibrous histiocytoma (dermatofibroma) arising on the face are reported. These neoplasms occurred frequently in females (24 female, 10 male) and showed a broad age range (12 to 85 years; mean: 43.6 years, median: 41 years). The neoplasms originated on the forehead (nine cases), the cheek (eight cases), the eyebrow (four cases), the temporal region (three cases), the nose (two cases), and the ear (one case); in seven cases the location face was given only. Five of 27 cases with follow-up information (median: 5 years) recurred locally; in one case four recurrences were excised within 8 years. The majority of cases extended into the subcutis and deep soft tissue including striated muscle (50% of cases). Histologically, only the minority of cases was composed entirely of histiocytoid and spindle-shaped tumor cells arranged in a storiform growth pattern. In many cases cellular fascicles and bundles of spindle-shaped tumor cells were noted in addition to classical morphological features of fibrous histiocytoma. A moderate mitotic rate (mean: 2.97 mitoses in 10 HPFs) was observed, and in few cases increased atypia was evident. Frank tumor necrosis and/or vascular invasion were not identified. Immunohistochemical studies revealed Factor XIIIa positivity in 13 out of 17, focal CD68 positivity in 6 out of 10, and alpha-smooth muscle actin positivity in 16 out of 19 cases tested. These lesions should be distinguished from dermatofibrosarcoma protuberans, including its fibrosarcomatous variant, leiomyosarcoma, and low-grade myofibroblastic sarcoma. Cases of fibrous histiocytoma of the face have to be excised with wider margins in comparison with examples of classical fibrous histiocytoma occurring on the extremities because of diffuse infiltration, involvement of deeper structures, and an increased rate of local recurrences.