Angiomatoid Malignant Fibrous Histiocytoma Research Articles

A simple Baker’s cyst? Tocilizumab remits paraneoplastic signs and controls growth of IL-6-producing angiomatoid malignant fibrous histiocytoma

A simple Baker’s cyst? Tocilizumab remits paraneoplastic signs and controls growth of IL-6-producing angiomatoid malignant fibrous histiocytoma

Reticular angiomatoid “malignant” fibrous histiocytoma—a case report with cytogenetics and molecular genetic analyses

Angiomatoid "malignant" fibrous histiocytoma is a rare sarcoma of low malignant potential that occurs most commonly in the extremities of children and young adults. Herein, we present a case of angiomatoid malignant fibrous histiocytoma with unusual histologic features arising in the mediastinum of an 80-year-old man. The tumor exhibited a reticular growth pattern and myxoid stroma. The tumor cells expressed epithelial membrane antigen and desmin. Cytogenetic analysis revealed the translocation t(2;22)(q33;q12). Molecular genetic analysis confirmed the rearrangement of the EWSR1 locus and the presence of the EWSR1/CREB1 fusion. This report expands the clinicopathologic spectrum of angiomatoid malignant fibrous histiocytoma and underscores the value of integrating morphologic, immunophenotypic, and molecular findings in the identification of its unusual morphologic variants.

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.

Fine‐needle aspiration cytology of angiomatoid malignant fibrous histiocytoma

Angiomatoid malignant fibrous histiocytoma (AMFH) is a rare, low-grade malignant mesenchymal neoplasm that affects mostly the extremities of children and young adults. Excisional surgery is the adequate treatment. The cytologic, immunocytologic, and histologic features noted in two patients having AMFH are presented. Cytologic smears showed histiocyte-like cells dispersed and in clusters, in close relation with eosinophilic mesenchymal fragments in a bloody background with lymphocytes. The tumor cells showed mild to moderate anisocariosis, often with nucleolus and vast, fragile cytoplasm. A fibroblastic-like spindle to ovoid cell population was also present in one patient. Immunohistochemical results are most consistent with myofibroblastic cell differentiation. When accompanied by adequate clinical information and ancillary techniques, a specific preoperative cytologic diagnosis is possible.

Fibroistiocitoma angiomatóide com metástase ao diagnóstico: relato de caso e revisão da literatura

Angiomatoid malignant fibrous histiocytoma is a rare and controversial entity, particularly as to its histogenesis. The tumor affects mostly the pediatric group, with an indolent clinical course and low rate of metastatization. In this report, the patient presented a lesion in the right foot and metastasis in an inguinal lymph node.

Angiomatoid “malignant” fibrous histiocytoma: A clinicopathologic study of 158 cases and further exploration of the myoid phenotype

Angiomatoid “malignant” fibrous histiocytoma (AMFH) has been considered to be a low-grade sarcoma of childhood, and, with its fibrous pseudocapsule, angiomatoid change, dense lymphoplasmacytic response, and proliferation of spindled or round cells, has been classified as a fibrohistiocytic neoplasm. We wanted to study the clinicopathologic and immunophenotypic features of a large number of these tumors and to especially further explore their myoid differentiation. Cases coded as AMFH from 1979 to 1995 were retrieved from the Soft Tissue Registry of the AFIP. Only cases that met the criteria for AMFH by light microscopy were included, a total of 158 cases. Immunohistochemistry was obtained on 98 cases. Clinical history on 92% of all cases revealed a gender ratio of 1.3 females: males, age range of 2 to 71 years, median size of 2.0 cm, and a distribution of extremities > trunk > head and neck, with 66% lesions occurring in areas of normal lymphoid tissue. All tumors with available margins were well-circumscribed. Eighty percent of cases had some degree of lymphoplasmacytic infiltration; 50% cases had pseudovascular spaces filled with blood. Fifty-two percent had predominantly round cell morphology; 48% had a predominantly spindle cell pattern. Desmin positivity was noted in 51% cases and occurred in both predominantly round cell and spindle cell tumors. Most of the desmin-positive cases with adjacent lymphoid infiltrate (67%) showed scattered similar, desmin-positive cells in the surrounding lymphoid infiltrate, adjacent to the tumor. Muscle-specific and smooth-muscle actins were seen in 14% cases. Heavy-caldesmon was strongly positive in 3%, and calponin was focally positive in 73% and extensively positive in 12% cases. MyoD1, myoglobin, and myogenin (myf4) were negative in all tumors studied. Forty-five percent of cases were positive for CD99; 52% of these had round cell morphology. Fifteen percent of cases were positive for KP-1. All tumors were positive for vimentin and negative for CD21, CD35, S100 protein, CD34, keratins 8 18 , and lysozyme. Clinical follow-up on 86 patients indicated that only 1 patient was alive with a local nodal metastasis (1% frequency of metastasis) within 1 year, and 2 others had local recurrence, all over a mean follow-up period of 6 years. The myoid, primarily myofibroblastic, phenotype of these lesions is supported by desmin, calponin, and occasional actin positivity. The occasional heavy-caldesmon and smooth muscle actin additionally suggest rare smooth muscle phenotype; however, lack of skeletal muscle markers indicate no relationship of AMFH to skeletal muscle tumors. The resemblance of these lesions to lymph nodes, clinically and morphologically, the finding of similar desmin positive cells in the adjacent lymphoid infiltrate, and the fact that 66% cases were found in sites of normal lymphoid tissue raise the possibility that some of these lesions may arise from or be related to myoid cells of lymphoid tissue. AMFH has an almost invariably benign behavior, but the 1% metastatic rate warrants its classification as low-grade “malignant.” The predominantly round cell, CD99-positive and desmin positive AMFH cases, respectively, should not be confused with Ewing's sarcoma/PNET or rhabdomyosarcoma, respectively.

Angiomatoid malignant fibrous histiocytoma: Report of an unusual case with highly aggressive clinical course

The authors report a case of angiomatoid malignant fibrous histiocytoma (AMFH), affecting a 9-year-old girl, with a highly aggressive clinical course. The tumor, noticed by the patient as a painless nodule in the dorsum of her left foot for 12 months, recurred 8 months after initial excision, and despite wide local reexcision, metastasized 4 months later to the liver and lung, where it grew at an alarming rate, to the extent of occupying the entire left hemithorax in a period of 10 weeks and killed the patient 14 months after initial excision. Review of the literature showed that the culminated rates of recurrence, metastasis, and mortality for AMFH were 23.2%, 8.7%, and 4.3%, respectively, indicating that it is definitely a malignant neoplasm with a potentially fatal outcome.

Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation.

The clinicopathologic features of 24 tumors showing perivascular myoid differentiation are described. These included tumors with histologic features of "infantile-type" myofibromatosis occurring in adult patients (8 cases), tumors with composite features of "hemangiopericytoma" and glomus tumor (9 cases), and tumors with a distinctive concentric perivascular proliferation of spindle cells (7 cases). Evidence of morphologic overlap among these groups suggests they are closely related neoplasms that form a single spectrum. Age of patients with lesions resembling infantile-type myofibromatosis ranged from 23 to 67 years (median, 37 years). Clinicopathologic manifestations of this disease included multicentricity (4 cases), local recurrence (3 cases), persistence of congenital lesions into adulthood (4 cases), and tumors that were multifocal within the confines of one anatomic region (7 cases). Histologically, all cases showed a biphasic pattern that consisted of fascicles of spindle cells with abundant eosinophilic cytoplasm that resembled smooth muscle, in addition to a population of more primitive spindled cells associated with a hemangiopericytomalike vascular pattern. Six cases showed reversal of the typical zonation seen in pediatric cases in that the primitive component surrounded the more mature fascicular areas. Also described are nine tumors with features that are intermediate between glomus tumor and hemangiopericytoma, which we have designated glomangiopericytoma. These tumors are characterized by prominent branching vessels lined by a single row of endothelial cells surrounded by epithelioid cells with a glomoid appearance. In other areas, the tumors showed typical hemangiopericytomatous foci similar to those in the myofibromatosis cases. The principal points of distinction were a lack of myoid nodules and an absence of small primitive cells with basophilic cytoplasm. Ages of these patients ranged from 17 to 78 years (median, 35 years). All tumors were located in the subcutaneous tissue and the superficial soft tissue of the extremities. Recurrence developed in one of six patients with follow-up information. The recurrent tumor had features of angiomatoid malignant fibrous histiocytoma. Finally, we describe a subset of tumors characterized by concentric periluminal proliferation of bland, round to ovoid cells, which we have designated as myopericytoma. Patient age ranged from 10 to 66 years (median, 40 years). All were located in subcutaneous and superficial soft tissue of distal extremities. One patient had two recurrences in 3 years after initial excision. Our study suggests that these three lesional groups comprise a histologic continuum of tumors that share clinical similarities and that, perhaps, are designated more appropriately as perivascular myomas. The relationship of this family of tumors to so-called hemangiopericytoma is discussed.

Unusual Soft Tissue Tumor with Features of Angiomatoid Malignant Fibrous Histiocytoma Composed of Bimodal CD34 and Factor XIIIa Positive Dendritic Cell Subsets: CD34 and Factor XIIIa in Angiomatoid MfH

CD34 and factor XIIIa (FXIIIa) antibodies delineate subsets of embryonic dendritic stromal stem cells that persist in adult mesenchyme. CD34 stains interstitial and adventitial dendritic cells that may function as multipotential precursor cells. FXIIIa+ dendrophages are tissue histiocytes active in tissue repair. Angiomatoid malignant fibrous histiocytoma (AMFH) is an enigmatic fibrohistiocytic tumor with limited vascular features. We examined an unusual soft tissue tumor from the nasolabial subcutis of a 57 year old man that showed histologic features of AMFH. Most of the tumor cells expressed CD34. 10-30% of the tumor cells were FXIIIa+ dendrophages. Sinusoidal areas were largely composed of FXIIIa+ cells that also expressed HLA-DR and CD68 suggesting macrophage differentiation. CD31 and Factor VIII antigen highlighted capillaries and single cells among the CD34+ tumor cells. The vessels had actin+ myopericytes and there were single actin+ tumor cells. Electron microscopy showed primitive dendritic cells and fewer histiocyte-like cells. The Ki 67 index was 15% including both FXIIIa+ and CD34+ cells. The patient is disease free three years after wide excision. We conclude that this AMFH-like neoplasm is a fibrohistiocytic tumor in which CD34+ fibroblast-like precursors and FXIIIa+ tissue dendrophages combine to build both sinusoidal tissue with endothelial and macrophage elements as well as capillary vascular tissue that is invested with myopericytes. Study of additional AMFH lesions from this standpoint is desirable.

Angiomatoid Malignant Fibrous Histiocytoma

This study reports the immunohistochemical, ultrastructural, and flow cytometric find ings of two cases of angiomatoid malignant fibrous histiocytoma that developed in the subcutis of the temple of a 10-year-old girl and the shoulder region of a 5-year- old boy. The neoplasm of the temple developed a local recurrence 6 months after the initial excision. Both patients were well and free from tumor at 24 months and 12 months, respectively. Immunohistochemically, many tumor cells of both cases posi tively expressed vimentin, desmin, HHF-35, and KP1. In one case many tumor cells also showed lectin binding for ulex europaeus agglutinin 1, and the other case pre sented ultrastructural features of immature endothelial cells. All tumors, including the recurrent lesion, were diploid. Divergent antigenic phenotypes along with many types of cells were considered; including myogenic cell, fibroblastic cell, and immature endo thelial cell. This study suggests that angiomatoid malignant fibrous histiocytomas show a wide range of pluripotential differentiation. Int J Surg Pathol 2(2):99-104, 1994

Evaluation of CD68 and Other Histiocytic Antigens in Angiomatoid Malignant Fibrous Histiocytoma

Immunohistochemistry for the histiocyte antigens CD68, L1, factor XIIIa, and S100 protein was performed on 19 cases of angiomatoid malignant fibrous histiocytoma (MFH) and for keratin, leukocyte common antigen, factor VIII-related antigen, muramidase, and desmin on six of these cases. Nine of 19 cases expressed the histiocyte differentiation antigen CD68, and in three cases expression was seen in greater than 90% of cells. Of the 19 cases studied, three contained rare S100-positive tumor cells, and two expressed factor XIIIa within tumor cells. In three of the six cases, rare desmin-positive tumor cells were identified. The neoplastic cells did not express the other antigens studied. CD68 was not detected in any of 12 cases of storiform-pleomorphic and myxoid MFH or in any of 24 cases of normal tissues or nonfibrohistiocytic mesenchymal tumors except for granular cell tumors. These results indicate that either the cells of angiomatoid MFH differentiate along lines of a tissue macrophage or alternatively, and perhaps more likely, are mesenchymal tumors with a phenotypic alteration, paralleling an enhanced capacity for phagocytosis and acquisition of lysosomes. In either event, the immunophenotype contrasts with conventional MFH and provides another independent set of observations in support of a separation of angiomatoid from conventional MFH.

Angiomatoid “malignant fibrous histiocytoma”: An immunohistochemical study indicative of myoid differentiation

Six cases of angiomatoid malignant fibrous histiocytoma (MFH), the rarest subtype of MFH, have been studied immunohistochemically using a broad panel of commercially available antisera in formalin-fixed, paraffin-embedded tissue in an attempt to define the pattern of differentiation shown by this unusual tumor. As has been reported in the more common types of MFH, no evidence of histiocytic differentiation was found. However, five cases strongly expressed desmin (DER-11) and two also expressed muscle actin (HHF 35). All tissues examined were negative for myoglobin and α-smooth muscle actin. These results provide good evidence for some sort of myogenic or possibly myofibroblastic differentiation in angiomatoid MFH. Given its clinicopathologically and immunohistochemically distinctive features, which are very different from the other variants of MFH, redesignation of angiomatoid MFH as a low-grade myogenic sarcoma of uncertain histogenesis is tentatively proposed. The new term angiomatoid myosarcoma is suggested.

Primary Sarcomas of the Major Salivary Glands

Sarcomas arising in the major salivary glands are rare. This article presents the clinicopathologic features of 11 patients with primary sarcomas of the parotid gland (eight men and three women aged 7 to 75 years; mean age, 42 years). The 11 sarcomas were histologically typified as follows: three malignant fibrous histiocytomas and two each of neurosarcomas, rhabdomyosarcomas, fibrosarcomas, and osteosarcomas. Four patients experienced disease recurrences, and seven patients developed metastases. Seven patients died of their sarcomas, with a mean survival time of 30 months. Four patients were alive from 5 to 12 years (one each with angiomatoid malignant fibrous histiocytoma, embryonal rhabdomyosarcoma, fibrosarcoma, and neurosarcoma). Prognosis correlated with size of the neoplasm, type of sarcoma, and histologic grade. In this respect, salivary gland sarcomas behaved in identical fashion to their soft-tissue counterparts.

Congenital, Infiltrating Giant-Cell Angioblastoma

This report describes histopathologic, immunohistologic, and ultrastructural features of a locally aggressive soft-tissue tumor present since birth in an upper extremity of an infant. Because of extensive infiltration of local structures, the lesion had to be treated by amputation. The outstanding histologic feature consisted of nodular cell clusters resembling inflammatory granulomas, often with giant, multinucleated cells. On the basis of our findings, these cellular aggregates were interpreted as distorted attempts at formation of vessels. This interpretation was strengthened by the more obviously vascular structure of the tumor in many areas. A high content of stromal cells positive for factor XIIIa and histocompatability antigen (HLA)-DR was a characteristic that the tumor shared in common with angiomatoid malignant fibrous histiocytoma. However, there were also important differences that singularize the tumor described in the present study. We could find no closely comparable precedent for a lesion with these characteristics in current treatises on infantile soft-tissue tumors.

Angiomatoid Malignant Fibrous Histiocytoma

Described by Enzinger in 1979, angiomatoid malignant fibrous histiocytoma is a distinctive tumor of adolescence and early adult life characterized by sheets of relatively bland rounded or spindled cells separated by areas of cystic hemorrhage and surrounded by a prominent inflammatory infiltrate and often a fibrous pseudocapsule. On the basis of the original 41 cases, the tumor has been considered a low-grade malignancy. We are reporting the clinicopathologic findings and follow-up information of 108 new cases of angiomatoid malignant fibrous histiocytoma to determine the long-term behavior of this tumor and whether various histologic features (atypia, mitoses, infiltrative borders, and inflammatory infiltrate) are useful in predicting outcome. Follow-up information was obtained in 94 (87%) cases. Local recurrences developed in 11 patients (12%), all of whom were cured by re-excision. The initial excision in all patients developing local recurrence appeared to be incomplete. Local recurrence was statistically associated with irregular tumor border and head and neck location. Five patients developed metastases. Four had only local metastases, which responded to surgery, whereas the fifth patient developed presumed pulmonary and cerebral metastases and died. The development of both local and distant metastases was correlated with invasion into the deep fascia or muscle but not to various histologic parameters such as mitotic rate and pleomorphism. We conclude that angiomatoid malignant fibrous histiocytoma is intrinsically a low-grade tumor, and assessment of various histologic parameters or grading provides little or no additional prognostic information. Because death from disease occurred in only one patient (1%) in our series, it seems reasonable to reclassify angiomatoid malignant fibrous histiocytoma with fibrohistiocytic tumors of intermediate malignancy rather than with the conventional malignant fibrous histocytoma, the majority of which are high-grade sarcomas.

Development of Vascular Neoplasia in Castlemanʼs Disease

Seven cases of vascular neoplasia arising within lesions of hypervascular follicular hyperplasia (HFH) fulfilling the criteria of Castleman's disease are described. The patients did not have evidence of acquired immunodeficiency syndrome or other immunologic disorders. The masses were solitary and located in the retroperitoneum (five cases), mediastinum (one case), and axilla (one case). Grossly, they measured up to 20 cm and had a variegated appearance. In each case two morphologically distinct processes were present: a mesenchymal spindle-cell neoplasm with evidence of vascular differentiation and Castleman's disease of hyaline vascular type. The two processes blended with each other, with the neoplasm appearing to be continuous with the interfollicular proliferation of small vessels that is typical of Castleman's disease. The lesions behaved aggressively in two cases, both patients having died with metastatic disease. This remarkable association may be viewed as a pathologic manifestation of the intimate functional relationship that exists between the immune and the vascular systems. Other probable examples of this relationship are systemic Castleman's disease associated with Kaposi's sarcoma, localized Castleman's disease associated with vascular hamartoma, histiocytoid hemangioma/angiolymphoid hyperplasia with eosinophilia, and (possibly) angiomatoid malignant fibrous histiocytoma. Perhaps these associations are mediated by the production of angiogenic factors by the activated lymphoid cells.

Multinodular Hemosiderotic Dermatofibroma

We describe a patient who developed a pigmented plaque on the anterior aspect of his right knee clinically mimicking angiokeratoma circumscriptum or malignant melanoma. Histopathological examination demonstrated a multinodular hemosiderotic dermatofibroma in which the cellular tumor islands were separated by areas of hyalinized collagen. Multinucleated giant tumor cells were found in the lumen of a medium-size vein underlying the tumor. The differential diagnosis of angiomatoid malignant fibrous histiocytoma, a tumor with clinical and morphologic similarities, is discussed.

Aneurysmal ("angiomatoid") fibrous histiocytoma in a child

A case of aneurysmal ("angiomatoid") fibrous histiocytoma (AFH) in a 12-year-old girl is presented with its unusual clinicopathologic features. The lesion had the full microscopic characteristics of AFH described in prior reports, but it also had some features that differed from the original description of the disorder, such as the involvement of subcutis, its occurrence in the scalp, and a documented history of minor trauma. The lesion clinically resembled the gross features of hemangioma. The experience in the present case raises the need for considering AFH as one of major differential diagnosis of nodular cutaneous tumors in children that simulate malignancy such as angiomatoid malignant fibrous histiocytoma and Kaposi's sarcoma.

Angiomatoid Malignant Fibrous Histiocytoma.: A Case Report

A case of angiomatoid malignant fibrous histiocytoma was presented. The tumor was unlike other variants of malignant fibrous histiocytoma because of prominent cystic change, haemorrhage and occurring in young adult. Our case was a 17-year-old boy who had been suffered from pyrexia and general fatigue. He was admitted to the medical ward in our hospital. The laboratory findings revealed only severe anemia, but the cause of pyrexia was not found. The patient was refered to us, orthopedists, with complainning of the soft part tumor of his right thigh. The pathologic examination by the biopsy showed malignant findings, then the tumor was removed widely with whole belly of the long adductor muscle.The tumor showed haemorrhagic cyst-like space with psuedocapsule macroscopically and the tumor was diagnosed microscopically as the angiomatoid malignant fibrous histiocytoma. His complaints and anemia were rapidly recovered after the operation. He has been followed for about five months, and no metastatic lesion and no local recurrence were found yet.

Angiomatoid Malignant Fibrous Histiocytoma Associated With Marked Bleeding Arising in Chronic Radiodermatitis

Enzinger and Weiss' classified angiomatoid malignant fibrous histiocytoma (AMFH) as a subtype of malignant fibrous histiocytoma (MFH). Clinically, AMFH manifests itself as a multinodular or multicystic, hemorrhagic mass.¹We saw a patient with a markedly bleeding, cystic tumor on chronic radiodermatitis of the lower abdomen. The risk of massive bleeding and its development on chronic radiodermatitis is reported herein. <h3>Report of a Case</h3> A 62-year-old woman was admitted to the hospital in June 1982, because of a bleeding, cystic tumor on chronic radiodermatitis of the lower abdomen. In 1966 she underwent total hysterectomy for cervical carcinoma and was put on a radiotherapy with a total of 8,000 rads of cobalt 60. She had noticed a fingertip sized nodule on chronic radiodermatitis six years prior. The nodule was symptomfree. It grew to be about the size of a table tennis ball during one year. It was reduced in size temporarily