Case Of Giant Cell Fibroblastoma Research Articles

Rare benign tumor in a prepubescent accessory breast: a case report

BackgroundPolymastia or accessory breast is a congenital condition seen in 1% of the population at birth and in 2–6% of the female population. The most common presentation is in the pubertal age group when secondary sexual characters begin to develop or during pregnancy and lactation. Giant cell fibroblastoma is an exceedingly rare benign soft tissue neoplasm in the pediatric age group, usually seen over the back and thigh. We report a case of giant cell fibroblastoma in accessory breast tissue in a 4-year-old, female child. This case is rare in its presentation at the age of 4 years with an enlarging accessory breast without any secondary sexual characters. Giant cell fibroblastoma in the accessory breast tissue is unreported yet.Case presentationA 4-year-old female child presented with a progressively enlarging accessory breast. A wide local excision of the accessory breast with underlying growth was done. The histopathological examination revealed a giant cell fibroblastoma within the breast tissue.ConclusionsEnlargement of an accessory or normal breast at a prepubescent age with the absence of secondary sexual characters should arouse suspicion of benign tumors in the breast tissue.

Very rare childhood tumor: Giant cell fibroblastoma

Giant cell fibroblastoma (GCF) is an intermediate grade, soft-tissue tumor of childhood with a high incidence of local recurrence. GCF merits attentive interpretation as they have been misdiagnosed as sarcoma in the past leading to erroneous remedies. Here, we are reporting the case of GCF in 13-month-old boy who presented with a painless swelling over the left gluteal region for the past 3 months.

Giant cell fibroblastoma

Giant cell fibroblastoma is a rare fibroblastic tumor occurring mainly in young patients. Back of thigh, inguinal region and chest wall are the most common sites of involvement. The tumor is characterized by a locally aggressive behavior with no reported metastatic potential. Microscopically, it is composed of spindle cells with a moderate degree of nuclear pleomorphism that infiltrate deep dermis and subcutis. A distinctive feature of this tumor is the presence of peculiar pseudovascular spaces lined by a discontinuous row of multinucleated cells. Features very similar to dermatofibrosarcoma protuberans can be seen. Immunohistochemically, it shows positive staining for CD34, while being negative for S-100 protein and vascular markers. The molecular changes are overlapping with dermatofibrosarcoma protuberans. A case of giant cell fibroblastoma will be presented illustrating the clinicopathological and molecular features of this tumor.

36. Giant cell fibroblastoma: A soft tissue tumour on a spectrum with dermatofibrosarcoma protuberans. report of a case

Background Giant cell fibroblastoma (GCF) is a soft tissue tumour which clinically, morphologically, immunohistochemi-cally and cytogenetically resembles dermatofibrosarcoma protu-berans (DFSP). Both GCF and DFSP occur preferentially in younger patients, have a male predominance and present as a slow growing painless mass in a truncal distribution in the dermis, subcutis or superficial skeletal muscle. Histologically, while DFSP contains hypercellular storiform areas, GCF is typically less cellular and contains pseudovascular spaces lined by giant cells, an onionskin-like perivascular lymphocytic infiltrate and fascial haemorrhage. Both tumours contain a t(17,22) translocation leading to generation of fusion protein COL1A1-PDGFRB. Both tumours have a predilection for local recurrence, and while DFSP rarely metastasises, GCF has not yet been reported to metastasise. Aim To highlight the diagnostic features of GCF. Methods We report a case of GCF in a 9-year-old boy who presented with an 11 cm non-tender slowly enlarging right axilla mass. Intra-operatively the lesion was within the pectoralis major muscle. We compare the morphology of GCF with that of DFSP and present supporting immunohistochemical and cytogenetic data. Results The tumour macroscopically appeared fleshy and haemorrhagic. Histologically the tumour exhibited varied cel-lularity containing pseudovascular spaces lined by large atypical cells. Similar atypical cells were noted in solid areas of the tumour. Immunohistochemical staining for CD34 was positive. Karyotyping revealed the presence of a t(17,22) translocation. Conclusion GCF is a soft tissue tumour that exists on a spectrum with DFSP. Awareness of the entity is important to avoid misdiagnosis as a malignancy.

Giant Cell Fibroblastoma of the Vulva at the Site of a Previous Fibroepithelial Stromal Polyp

Giant cell fibroblastoma (GCF) is an unusual soft tissue tumor, occurring predominantly in infants and children, and rarely in adults. Giant cell fibroblastoma develops de novo in the dermis or subcutis with a predilection for the extremities, the abdominal and chest walls, umbilical and inguinal regions. A GCF arose at the same site (labium majus of vulva) as a previous cellular fibroepithelial stromal polyp in a 28-year-old woman. We report a case of GCF of the vulva, an unreported site.

Giant cell fibroblastoma: an update and addition of 86 new cases from the Armed Forces Institute of Pathology, in honor of Dr. Franz M. Enzinger

A quarter of a century ago at the International Academy of Pathology in Boston, Mass, Drs Enzinger and Shmookler's seminal abstract on giant cell fibroblastoma (GCF) included 20 GCFs on the back and thigh of mostly male children. These tumors involved dermis and subcutis, and had parallel fascicles of wavy uniform spindled cells with wiry collagen, dense sclerosis, and gaping spaces with scattered and rimming pleomorphic giant cells. EM suggested fibroblastic phenotype. All cases had benign behavior, but almost half recurred. The caveat was mistaking this tumor for a malignancy. In 1989, Drs Enzinger, Shmookler, and Weiss published this abstract as 28 cases from the AFIP (1960-1981), including 4 adults up to 55 years old. They proposed a relationship of this childhood tumor to dermatofibrosarcoma protuberans (DFSP). Since these original descriptions of GCF, there has been additional immunohistochemical and molecular support for a relationship between DFSP and GCF. We reviewed additional AFIP cases of GCF since 1981, in honor of Dr Enzinger. These new cases included 60 males and 26 females, whose ages ranged from 6 months to 62 years (median, 6 years; 62%, younger than 10 years; 77%, younger than 20 years; and only 10 patients were older than 40 years). Thirty-nine GCF cases with evaluable epidermis were observed to be protuberant, one with superficial ulceration. Most cases were dermal and subcutaneous, 3 purely dermal, and 5 involved superficial skeletal muscle. Almost all cases demonstrated a honeycomb pattern, and several, a parallel pattern of infiltration. Several cases spared adnexa. Pure GCF areas ranged from solid and collagenized to angiectoid and myxoid, the latter with small to large cystlike spaces. Most cases were relatively hypocellular, except one case with more atypia and mitotic activity. GCF demonstrated myoid whorls in 2 cases, a feature previously described in DFSP. Most remarkable is the peculiar perivascular lymphocytes in an onionskin pattern in GCF, not observed in DFSP. Furthermore, histologic intralesional hemorrhage seems to be common in GCF, particularly near the fascia. Fourteen of our 86 cases demonstrated 5% to 70% (median, 20%) dense nongiant cell storiform areas, interpreted as hybrid GCF-DFSP. Three of these cases demonstrated hypercellular DFSP. One hybrid case had fibrosarcomatous transformation. Two cases of pure GCF recurred as a hybrid tumor with DFSP areas, one of these with hypercellular DFSP. In all but one case, the DFSP was adjacent to GCF with an abrupt transition. Most cases studied were positive for CD34 (more intense in DFSP than relatively hypocellular GCF areas) and negative for smooth muscle actin, desmin, HMB-45, keratin, and S100 protein. GCF is exactly clinically and morphologically the same as Dr Enzinger and colleagues originally described it. Additional observations of marked perivascular and onionskin-like chronic inflammation and consistent hemorrhage may aid in the diagnosis of this previously well-described tumor. Collectively, we now have even more convincing morphologic, immunophenotypic, and molecular evidence that GCF is on a spectrum with DFSP.

Fine-Needle Aspiration Biopsy Features in a Case of Giant Cell Fibroblastoma of the Chest Wall

Giant cell fibroblastoma is an unusual tumor of childhood, primarily occurring in the superficial soft tissues. We describe the fine-needle aspiration biopsy features of a case of giant cell fibroblastoma of the chest wall in a 3-year-old child. The aspirates comprised bland spindle to oval cells entrapped in a metachromatic matrix, accompanied by rare multinucleated giant cells with wreathlike nuclei. Although we were unable to render a definitive diagnosis on fine-needle aspiration biopsy, surgical resection of the mass established the diagnosis of giant cell fibroblastoma. We review the distinctive cytologic features of some common soft tissue tumors arising in this age group that may give rise to a diagnostic conundrum on fine-needle aspiration biopsy.

Giant-Cell Fibroblastoma with Pigmented Dermatofibrosarcoma Protuberans Component

A unique case of giant-cell fibroblastoma that contained a pigmented dermatofibrosarcoma component (Bednár tumor) is described. The melanin pigmented dendritic cells found in the dermatofibrosarcoma component were round or spindle-shaped and had bipolar extensions. They were S-100 protein positive. Our case indicates the close histogenetic relationship between giant-cell fibroblastoma and dermatofibrosarcoma protuberans. The occurrence of the pigmented melanin-laden dendritic cells in our tumor further substantiates the theory that Bednár tumor is a pigmented variant of dermatofibrosarcoma protuberans.

Giant cell fibroblastoma

A report of a 1 1/2-year-old male child diagnosed as a case of giant cell fibroblastoma is described and the available literature on this neoplasm is reviewed. The tumor consists of a mixture of spindle cells and multinucleated giant cells in a myxoid or collagenous background. The pathological findings and clinical course suggest that these tumors be classified with fibromatoses of childhood. Removal of the tumor with wide excision is sufficient and these patients do not require any form of chemotherapy.

Giant Cell Fibroblastoma Recurring as Dermatofibrosarcoma Protuberans

A case of giant cell fibroblastoma in an 8-year-old child recurred locally 15 years later with the typical picture of dermatofibrosarcoma protuberans. This sequence of events suggests that giant cell fibroblastoma is a juvenile form of dermatofibrosarcoma protuberans.

Giant Cell Fibroblastoma A Case Report

A case of giant cell fibroblastoma occurring in the knee of a 16-month-old girl is reported. The ill-defined subcutaneous tumor measuring 2 x 2 cm was composed of diffusely proliferating spindle-shaped tumor cells with scattered, atypical multinucleated giant cells in a myxoid or collagenous background and irregularly branching sinusoid-like tissue spaces. In addition to floret-type giant cells, a few osteoclast-like giant cells were present in a cellular area where tumor cells were focally arranged in a storiform pattern. Immunohistochemically, the tumor cells gave positive reactions for only vimentin and actin. In spite of the high recurrence rate of this type of tumor, the course of the patient after excision of the tumor has been uneventful. It is important to distinguish this rare, peculiar fibrous tumor from other soft tissue tumors including some sarcomas.

Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans.

A clinicopathologic analysis of 28 cases of giant cell fibroblastoma (GCF), a rare mesenchymal tumor occurring predominantly in the first decade of life, is presented. This disease presented as a painless, slowly enlarging, subcutaneous mass. The tumor recurred locally in 47% of the patients; however, metastasis was not detected. On microscopic examination, GCF showed an unique combination of spindle cell patterns, pleomorphic and multinucleated giant cells, myxoid areas, and distinctive sinusoid-like spaces. This unrecognized histomorphologic picture led to a misdiagnosis of sarcoma in 40% of the cases. The histogenesis of this lesion remains uncertain; however, based on both clinical and morphologic similarities, it is proposed that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP).