Fibrohistiocytic Tumor Of Intermediate Malignancy Research Articles

Giant Cell Tumor of Soft Tissue Masquerading as a Fibroma - A Rare Clinical Entity

Giant cell tumor of the soft tissue (GCT-ST) is a rare and distinctive entity, separate from its bony counterpart, giant cell tumor of bone. This primary soft tissue tumor is often encountered in middle-aged individuals but rarely in children, with common sites including superficial soft tissue of the extremities. GCT-ST typically presents as a painless, slowly growing mass, emphasizing the importance of complete excision to prevent recurrences and metastasis. We present a case of GCT-ST in a 7-year-old boy with a nodular swelling on the right hip, initially suspected to be a fibroma. Histopathological examination revealed characteristic features of GCT-ST, including spindle and stellate cells, multinucleated giant cells, and haemosiderin-laden macrophages with metaplastic osseous tissue. This case adds to the limited literature on GCT-ST, highlighting its diverse clinical presentations and challenging diagnostic nature. The discussion includes a review of reported cases of GCT-ST, which have shown varied clinical presentations and outcomes. Histologically, GCT-ST resembles a giant cell tumor of bone, with positive staining for CD68, SMA, Vimentin, and p63. Imaging studies typically reveal a solid hemorrhagic mass. Differential diagnoses include benign and malignant soft tissue tumors rich in giant cells. Keywords: Giant Cell Tumor of Soft Tissue (GCT-ST), Rare, Fibro-Histiocytic Tumor Of Intermediate Malignancy, Soft Tissue Tumor, Rare Tumors In Children.

Primary breast stromal sarcoma: A rare case report

Primary sarcoma of the breast comprises less than 1% of all the breast malignancies. It is a locally aggressive neoplasm with only a few hundred cases published till date. The index case is being reported due to rarity of this entity. Breast sarcoma should always be considered whenever spindle cells are seen in the histological sections. The main diagnostic confusion arises with malignant phyllodes tumor and metaplastic carcinoma from which distinction is important from prognosis as well as treatment point of view. Herein, we report a case of a 47-yr-old female, who presented with a right-sided breast lump which was diagnosed as suspicious of carcinoma breast on fine needle aspiration (FNAC). Thereafter, modified radical mastectomy was performed, and on histopathology, a possibility of fibro-histiocytic tumor of intermediate malignancy was rendered, which was subsequently confirmed by immunohistochemistry (IHC).

The Immunological Roles of Periostin/Tumor-Associated Macrophage Axis in Development of Dermatofibrosarcoma Protuberans.

Dermatofibrosarcoma protuberance (DFSP) is a fibrohistiocytic tumor of intermediate malignancy characterized by slow infiltrative growth and a high tendency to recur locally. Periostin is involved in modulating cell function and inducing the production of proinflammatory cytokines, chemokines, and matrix metalloproteinases (MMPs) from tumor-associated macrophages (TAMs) to promote fibrosis and tumor growth. This study aimed to examine the cancer stroma of DFSP, focusing on TAMs-related proteins and MMPs. Using immunohistochemical staining and DNA microarray database, we evaluated periostin, CD163, CD206, MMP1 and MMP12 in 10 cases of DFSP and dermatofibroma. Dense deposits of periostin as well as a substantial number of CD163+ TAMs were detected at the peripheral areas of DFSP. Moreover, MMP1 and MMP12, that were selected by using a DNA microarray database of monocyte-derived macrophages, were observed in the TAMs-detected area. Increased levels of MMP1 and MMP12 on TAMs in the peripheral areas of DFSP might contribute to local invasion.

Atrophic Dermatofibrosarcoma: the importance of clinical suspicion

Dermatofibrosarcoma protuberans is an uncommon and relapsing fibrohistiocytic tumor of intermediate malignancy. Its rarest variant is known as atrophic, morphea type or dermatofibrosarcoma non protuberans and its clinical diagnose is difficult. There are not well established dermatoscopic criteria, so the diagnosis is histopathologic. There are advances in immunohistochemical and genetic that help the diagnosis and complete surgical excision. It is reported a case of atrophic dermatofibrosarcoma in a 27 years patient ant it is reviewed the clinical, dermoscopic, histological and genetic aspects

Dermatofibrosarcoma Protuberans in Childhood

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age.The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence.This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options.

Dermatofibrosarcoma protuberans en la infancia

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age.The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence.This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options.

Plexiform fibrohistiocytic tumor—report of one case with regional lymph node metastasis

A plexiform fibrohistiocytic tumor (PFT) is a rare mesenchymal neoplasm primarily occurring in children and young adults. PFTs have been classified as fibrohistiocytic tumors of intermediate malignancy because of the high local recurrence rate and possible lymph node and distant metastasis. Histologically, PFTs are poorly demarcated dermal-to-subcutaneous tumors composed of small nodules or cellular clusters with a characteristic plexiform arrangement. We report a 17-year-old girl presenting with a nasal root tumor and concurrent cervical lymphadenopathy. Light microscopy and immunohistochemical findings were compatible with a PFT, for both the nasal root tumor and the cervical lymph nodes. Under the diagnostic impression of a PFT with neck lymph node metastasis, the patient underwent wide excision of the primary tumor and cervical lymph node dissection, followed by concurrent chemo-radiotherapy. During a 4-year follow-up, no evidence of recurrence was noted. We also review the previously published cases of PFTs with metastasis.

Polypoid Giant Cell Tumor of the Skin

Giant cell tumor of soft tissue is a rare neoplasm named for its resemblance to giant cell tumor of bone. According to World Health Organization classification of soft tissue tumors, it belongs to the category of the so-called fibrohistiocytic tumors of intermediate malignancy, characterized by frequent local recurrences unless widely excised, but only rarely metastases to lymph nodes and lungs. Cutaneous presentation is extremely rare. We described a case occurring in a 79-year-old woman who presented with a nodular, polypoid, ulcerated lesion on the paranasal region. The histomorphological features were consistent with the diagnosis of primary giant cell tumor of the skin. Clinical informations and immunohistochemistry are useful in distinguishing this neoplasm from other neoplastic and reactive lesions of the superficial soft tissues containing giant cells.

Angiomatoid Fibrous Histiocytoma in a Six‐Year‐Old Child

We report a case of AFH presenting as an asymptomatic subcutaneous nodule on the arm of a 6-year-old boy. AFH is a fibrohistiocytic tumor of intermediate malignancy. Predominantly seen in children and young adults, AFH presents as a deep dermal or subcutaneous nodule usually on the extremities. The histology is characterized by a fibrous capsule, surrounding lymphocytic infiltrate and blood-filled cystic spaces lined by flattened tumor cells. AFH cells express desmin, epithelial membrane antigen, and CD 68 in over 60% of cases; they are negative for myogenin, MYOD1, and endothelial markers. Rate of local recurrence is 2% to 20%. The metastatic rate is 1%. Management is with wide surgical excision and careful follow-up.

Plexiform Fibrohistiocytic Tumor in Three Children

Plexiform fibrohistiocytic tumor is a very rare fibrohistiocytic tumor of intermediate malignancy. It can occur at any age but is more prevalent in children and in young adults. Here we present the clinicopathologic findings of three girls with this tumor. The patients were 8 months, 14 months, and 7 years of age. They each presented with a solitary, nontender, subcutaneous nodule or plaque. Light microscopy and immunohistochemical study findings were compatible with plexiform fibrohistiocytic tumor. We also review the previously published cases in the English-language literature.

Cutaneous and Subcutaneous Fibrohistiocytic Tumors of Intermediate Malignancy

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 morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy characterized by a distinctive storiform growth pattern and frequent local recurrences. In this study, we retrospectively reviewed 48 cases of DFSP diagnosed at the Cleveland Clinic Foundation between 1970 and 1999 to determine the prevalence of morphologic variations including the presence of giant cell fibroblastoma (GCF)-like areas, multinucleated giant cells, hypercellular zones and fibrosarcomatous change. The cohort consisted of 42 patients (20 males, 22 females) with a median age at diagnosis of 40 years (range: 10-73 years). Forty-one primary tumors and seven recurrences were evaluated from these 42 patients. Tumor sites included the trunk (22 cases), head and neck (8 cases), upper extremities (7 cases) and lower extremities (6 cases). GCF-like areas were identified in seven (14.6%), multinucleated giant cells in ten (20.8%), hypercellular zones in 12 (25%) and fibrosarcomatous change in six (12.5%) cases, respectively. Combinations included giant cells and GCF-like areas (two cases), giant cells and hypercellular zone (two cases), and GCF-like areas and hypercellular zones (one case). Our findings suggest that DFSP has a wider range of morphologic features, including GCF-like areas, multinucleated giant cells, hypercellular zones and fibrosarcomatous change, than has been previously recognized in the literature.

A Pediatric Case of Dermatofibrosarcoma Protuberans: An Immunohistochemical Study

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that usually occurs in early or mid-adult life as a nodular cutaneous mass. DFSP has rarely been reported during childhood or at birth. We report a case of childhood DFSP that illustrates the usefulness of immunohistochemical analysis in the differential diagnosis between DFSP and other fibrohistiocytic proliferations occurring in the skin. A prompt and correct diagnosis is very important in order to ensure appropriate treatment and to prevent local recurrences.

Dermatofibrosarcoma protuberans: an analysis of proliferative activity, DNA flow cytometry and p53 overexpression with emphasis on its progression.

The aim of this study is to evaluate the degree and spectrum of malignancy of dermatofibrosarcoma protuberans (DFSP) in the aspect of proliferative activity, flow cytometric DNA analysis, and p53 immunoreactivity. Twenty-three tumors from 19 cases of DFSP including three cases of DFSP with fibrosarcomatous areas (DFSP-FS) were studied in comparison with its allied fibrohistiocytic tumors; that is, dermatofibroma (DF; 46 cases), fibrosarcoma (FS; four cases), and malignant fibrous histiocytoma (MFH; 11 cases). MIB-1 labeling index (LI) of DFSP was significantly higher than that of DF and was lower than those of FS and of MFH. In ordinary DFSP, the recurrent tumors exhibited significantly higher MIB-1 LI than that of the primary tumors, whereas the primary tumors showed almost the same proliferative activity of DF. DFSP-FS tended to have a higher proliferative activity than DFSP without FS-area (ordinary DFSP). In five of 19 cases of DFSP, aneuploidy (near-diploidy) was found in four recurrent and one primary tumors. Immunohistochemical p53 overexpression was found in three of 19 cases of DFSP which also showed higher proliferative activity and aneuploidy. All cases of DF were immunohistochemically negative for p53, but most of the cases of FS and MFH were positive. Although DFSP has been classified in a category of fibrohistiocytic tumor of intermediate malignancy, the recurrent DFSP, DFSP-FS, and DFSP with aneuploidy and/or p53 overexpression could be a subgroup of DFSP with more aggressive clinical behavior than ordinary primary ones.

Malignant fibrous tumors.

Dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and malignant fibrous histiocytoma (MFH) are rare soft tissue sarcomas of intermediate to high-grade malignancy that the dermatologist must evaluate and treat. DFSP is a fibrohistiocytic tumor of intermediate malignancy characterized by aggressive local growth and propensity to recur after surgical excision. AFX is a superficial malignant tumor that arises on sun-exposed surfaces and is characterized by local aggressive behavior. MFH is the most aggressive of the fibrohistiocytic tumors with a high local recurrence rate and significant metastatic rate usually associated with a poor prognosis. This article reviews the epidemiology, pathogenesis, clinical presentation, histology, management, prognosis, and follow-up of these malignant tumors.

Nucleolar Organizer Regions in Soft Tissue Sarcomas

Argyrophilic proteins of nucleolar organizer regions (AgNORs) were studied in a series of 65 sarcomas of the soft tissues and 2 cases of fibrohistiocytic tumors of intermediate malignancy. The numbers of AgNORs per nucleus and the size of AgNORs were determined and compared with pathomorphologic parameters such as grading of malignancy, cellularity and tumor diameter. The main finding of this study was that AgNOR counts showed significant differences between low-grade malignant (G 1) and high-grade malignant (G 3) tumors predominantly based on a correlation with the frequency of mitosis. All the other criteria of tumor grading (nuclear pleomorphism, differentiation, amount of necrosis), tumor diameter and cellularity showed no differences with regard to AgNOR counts. Though it was observed that tumor giant cells possessed predominantly coarse granular AgNORs, no correlation was found between AgNOR size and any of the parameters investigated. In spite of a clear statistically significant result gained by an evaluation of all cases, a heterogeneity of AgNORs in relation to mitotic activity within a few histological tumor types could be observed, making it difficult to suggest the determination of AgNORs as a parameter with general validity for all soft tissue sarcomas.

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.