A malignant fibrous histiocytoma is a rare pleomorphic tumor of the soft tissues. Primary retroperitoneal involvement of a malignant fibrous histiocytoma is very rare and often diagnosed in an advanced stage as it has no specific symptoms. Herein, a case of a malignant fibrous histiocytoma involving the retroperitoneum is reported, which was incidentally found in a 74-year-old male, who complained of pain in the right abdomen and thigh.
Abdomen ; Aged ; Histiocytoma, Benign Fibrous ; Histiocytoma, Malignant Fibrous* ; Humans ; Male ; Retroperitoneal Space ; Soft Tissue Neoplasms ; Thigh

PURPOSE: We wanted to retrospectively determine the accuracy of an ultrasonographic diagnosis of superficial soft tissue masses in the extremities by using the histologic results as the reference standard. MATERIALS AND METHODS: From January 2005 to June 2010, 154 patients with soft tissue masses in the extremities and who underwent ultrasonographic evaluation followed by biopsy or resection were retrospectively evaluated. The ultrasonographic and histologic diagnoses of the soft tissue masses were lipoma, ganglion cyst, hemangioma, neurogenic tumor, giant cell tumor of the tendon sheath, epidermoid cyst, fibroma, glomus tumor, Baker's cyst and neurofibromatosis. RESULTS: Out of 154 patients, 114 (74%) patients showed concordance between the histologic diagnosis and the ultrasonographic diagnosis, and the remaining 40 (26%) patients did not. The diagnostic accuracy of each soft tissue mass was 95% for lipoma, 83% for ganglion cyst, 75% for hemangioma, 72% for neurogenic tumor, 50% for giant cell tumor of the tendon sheath, 43% for epidermoid cyst, 33% for fibroma and 100% each for glomus tumor, fibromatosis and Baker's cyst. Aside from these tumors, there were also sarcoma, malignant melanoma, elastofibroma, Kimura disease and pilomatricoma. Among the cases that showed discordance between the histologic diagnosis and the ultrasonographic diagnosis, three of them were notable; pilomatricoma being misdiagnosed as dermatofibroma protuberans, angiolipoma being misdiagnosed as vascular leiomyoma and malignant fibrous histiocytoma being misdiagnosed as a malignant soft tissue mass. CONCLUSION: The accuracy of an ultrasonographic diagnosis for soft tissue masses in the extremities varies greatly according to each type of mass. Lipoma, ganglion cyst, hemangioma, glomus tumor, neurogenic tumor and Baker's cyst showed a relatively high rate of concordance between the ultrasonographic diagnosis and the histologic diagnosis, but epidermoid cyst and fibroma showed a relatively lower rate of concordance.
Angiolipoma ; Angiolymphoid Hyperplasia with Eosinophilia ; Angiomyoma ; Biopsy ; Epidermal Cyst ; Extremities ; Fibroma ; Ganglion Cysts ; Giant Cell Tumors ; Glomus Tumor ; Hemangioma ; Histiocytoma, Benign Fibrous ; Histiocytoma, Malignant Fibrous ; Humans ; Lipoma ; Melanoma ; Pilomatrixoma ; Popliteal Cyst ; Retrospective Studies ; Sarcoma ; Soft Tissue Neoplasms ; Tendons

OBJECTIVETo study the pathological characteristics, diagnosis and differential diagnoses of hemangiopericytoma-solitary fibrous tumor with giant cells.

METHODSPathological characteristics of seven cases of orbital and extraorbital hemangiopericytoma-solitary fibrous tumors with giant cells were evaluated by HE and immunohistochemistry (EnVision method).

RESULTSTwo cases were located in the orbit, one of which had recurred. Five cases were located in the extraorbital regions. Histologically, the tumors were well-circumscribed and composed of non-atypical, round to spindle cells with collagen deposition in the stroma. The tumors had prominent vasculatures and in areas, pseudovascular spaces lined by multinucleated giant cells lining which were also present in the stroma. Immunohistochemically, both neoplastic cells and multinucleate giant cells expressed CD34. Seven patients underwent tumor excision and were well and without tumor recurrence upon the clinical follow-up.

CONCLUSIONSHemangiopericytoma-solitary fibrous tumor with giant cells is an intermediate soft tissue tumor. It typically involves the orbital or extraorbital regions. Histologically, the tumor should be distinguished from giant cell fibroblastoma, pleomorphic hyalinzing angiectatic tumor of soft part and angiomatoid fibrous histiocytoma.

12E7 Antigen ; Adult ; Antigens, CD ; metabolism ; Antigens, CD34 ; metabolism ; Cell Adhesion Molecules ; metabolism ; Dermatofibrosarcoma ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangiopericytoma ; metabolism ; pathology ; surgery ; Histiocytoma, Benign Fibrous ; pathology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Orbital Neoplasms ; metabolism ; pathology ; surgery ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Soft Tissue Neoplasms ; pathology ; Solitary Fibrous Tumor, Pleural ; metabolism ; pathology ; surgery ; Solitary Fibrous Tumors ; metabolism ; pathology ; surgery ; Young Adult

Arm ; Fibroma ; Histiocytoma, Malignant Fibrous ; Humans ; Soft Tissue Neoplasms

OBJECTIVETo study the clinicopathologic features, immunophenotype and differential diagnosis of angiomatoid fibrous histiocytoma (AFH).

METHODSThe clinicopathologic features of 5 cases of AFH were analyzed. Immunohistochemical study was carried out and the literature was reviewed.

RESULTSThere were a total of 3 males and 2 females. The average age of patients was 21.4 years old. The average duration of symptoms was 13 months. The patients primarily presented with a slowly enlarging painless deep dermal or subcutaneous mass. The mass was located in the head and neck region in 3 cases, elbow in 1 case and foot in 1 case. The patients underwent complete resection of the tumor, with no adjuvant chemotherapy and/or radiotherapy given. During a period of follow up for 10 to 29 months, all of them had no recurrence or distant metastasis. Gross examination showed that the tumor was well-circumscribed and had a grey-colored cut surface, with focal hemorrhagic cystic changes. The average tumor dimension was 1.9 cm. Histologically, the tumor was composed of histiocytoid or spindly cells arranged in nodular pattern. Fibrillary neuropil-type intercellular material was identified in all cases and a fibrous pseudocapsule surrounded by lymphocytes and plasma cells was demonstrated in 3 cases. Immunohistochemical study showed that all of them were positive for vimentin and negative for S-100 protein, pan-cytokeratin, CD34 and CD31. Three of the cases expressed desmin and CD68. Two cases were epithelial membrane antigen and CD99-positive.

CONCLUSIONSAFH is a rare tumor of intermediate malignant potential. Definitive diagnosis requires thorough histologic examination and clinical correlation. Immunohistochemistry is also helpful for diagnosis and differential diagnosis. Wide local excision with post-operative follow up is the main modality of treatment.

Adolescent ; Adult ; Aneurysm ; metabolism ; pathology ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Chemotherapy, Adjuvant ; Child ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; surgery ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Male ; Radiotherapy, Adjuvant ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo investigate the clinical pathological features of fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP).

METHODSThe clinical history, histopathological features and immunohistochemical characteristics were analyzed in twelve cases of FS-DFSP from January 1997 to February 2011, and related literature were reviewed.

RESULTSAge of the patients (2 females, 10 males) at diagnosis ranged from 41 to 70 years (mean 53 years). Among the 12 cases of FS-DFSP, 9 cases aroused in recurrent ordinary DFSP. Histologically, FS areas in FS-DFSP were characterized by a fascicular and highly cellular histology, frequently showing a characteristic herringbone pattern. FS-DFSP showed diminishment of CD34 staining in FS areas. The labeling index of Ki-67 was much higher in the FS areas (10%-40%) than that in the conventional DFSP areas (2%-5%). All the patients were treated by operation with local excision or wide excision. Postoperative radiotherapy and chemotherapy was administered in two cases respectively. Follow-up information in 9 of 12 patients (9 to 86 months) revealed local recurrence in 6 patients. Distant metastases were seen in two patients. One patient was died in the follow up period.

CONCLUSIONSFS-DFSP is a rare and unique subtype of DFSP and is associated with significant elevated risk of both local and distance metastasis, usually followed by poor outcome. Compared to ordinary DFSP as a borderline neoplasm, FS-DFSP should be considered as a malignant tumor.

Adult ; Aged ; Antigens, CD34 ; metabolism ; Chemotherapy, Adjuvant ; Dermatofibrosarcoma ; metabolism ; pathology ; therapy ; Diagnosis, Differential ; Female ; Fibroma ; pathology ; Fibrosarcoma ; metabolism ; pathology ; therapy ; Follow-Up Studies ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Radiotherapy, Adjuvant ; Retrospective Studies ; Skin Neoplasms ; metabolism ; pathology ; therapy

Perineurioma is a rare benign peripheral nerve sheath tumor, composed uniformly of perineurial cells. Soft tissue perineurioma primarily arises within the subcutaneous tissue of extremities and trunk as a painless solitary nodule, and should be distinguished from dermatofibroma, neurofibroma, dermatofibrosarcoma protuberans, meningioma and so on. A 25 year-old female is presented with three small asymptomatic papules on the third left finger which were found 3 years ago. Punch biopsy was performed on all of the papules. Microscopic examination demonstrated well-demarcated tumor within dermis, and proliferation of spindle cells with wavy nuclei and elongated bipolar cytoplasmic process, arranged in a whorled pattern. According to immunohistochemical analysis, the tumor cell showed positivity for epithelial membrane antigen, but negativity for S-100 protein, factor XIIIa, CD34, and smooth muscle actin. The diagnosis of soft tissue perineurioma was being made. We report this rare case of perineurioma presented as multiple papules localized within dermis of the digit.
Actins ; Biopsy ; Cytoplasm ; Dermatofibrosarcoma ; Dermis ; Extremities ; Factor XIIIa ; Female ; Fingers ; Histiocytoma, Benign Fibrous ; Humans ; Immunohistochemistry ; Meningioma ; Mucin-1 ; Muscle, Smooth ; Nerve Sheath Neoplasms ; Neurofibroma ; Peripheral Nerves ; S100 Proteins ; Subcutaneous Tissue

A benign fibrous histiocytoma (BFH) is one of the fibrohistiocytic groups of soft-tissue tumors for which spinal involvement is extremely rare. To the best of our knowledge, most spine-originating BFHs are bone tumors. We report the first case of BFH occurring in the intraspinal extradural space on the lumbar spine. A 66-year-old female presented with severe claudication symptom. The preoperative magnetic resonance images showed a huge intraspinal, extradural, thecal-sac-compressing soft-tissue tumor that extended along the right L5 root to the neural foramen. The tumor was a relatively well-marginated, inhomogeneous soft-tissue mass with some fluid-containing cystic portions that were well enhanced by the gadolinium contrast dye. After a total facectectomy, the tumor was removed completely. The patient had a good neurological recovery without complications, and no recurrence was noted at the 6-month follow-up.
Aged ; Female ; Follow-Up Studies ; Gadolinium ; Histiocytoma ; Histiocytoma, Benign Fibrous ; Humans ; Lumbar Vertebrae ; Magnetic Resonance Spectroscopy ; Recurrence ; Soft Tissue Neoplasms ; Spine

BACKGROUND: The relationship and differentiation among various dendritic cells of the dermis are unclear. Recently it has hecome possible to identify different subpopulat,ions of dermal dendritic cells using anti-CD34 and anti-factor XIIIa antibodies. OBJECTIVE: To elucidate which cell types the fibrous dermal turnors consist of we compared the staining patterns of these antibodies as well as of anti-Mac 387 antibody which are labeled as inflammatory cells of the monocyte-macrophage lineage. METHODS: Tumors studied included dermatofibrosarcoma protuberans(DFSP, n=2), dermato-fibroma(n=22), neurofibroma, n=27), acrochordon(n=15), keloid, hypertrophic scar(n=10), juvenile xanthogranuloma(n=1, and malignant fibrous histiocytoma (MFH, n=1). We performed immunoperoxidase staining(AUSC technique) with polyclonal anti FXIIIa antibody, monoclonal anti-CD34 antibody, and monoclonal anti-Mac 387 antibody on the formalin-fixed-paraffin-embedded tissue specimens of these fibrous tumors. The intensity of staining was graded as negative, weakly staining, or strongly stainiring. RESULTS: FXIIIa reactivity was strongly present in dendritic and spindle-shaped cells of all dermatofibromas and some nurofibromas(11 of 27 specimens), but absent from the other fibrous tumors. Among these tumors, one of the two DFSPs was uniquely expressed CD34. Dendritic and spindle-shaped cells within tiese tumors were MAC 387 negative. In inflammatory conditions, variable numbers of MAC 38 positive cells were observed, corresponding to histiocytes and mac-rophages, but the labeling of ipithelioid cells and multinucleated foreign body giant cells were variable. CONCLUSION: The findings of significant numbers of FXIIIa positive cells in dermal fibrous tumors studied suggest that thet may be diagnostic utility associated with the use of this antit)ody. In addition, CD 34 expression by the tumor cells can be an extremely useful marker in establishing a definitive diagnosis of IFSP.
Antibodies ; Dendritic Cells ; Dermatofibrosarcoma ; Dermis ; Diagnosis ; Factor XIIIa* ; Giant Cells, Foreign-Body ; Histiocytes ; Histiocytoma, Benign Fibrous ; Histiocytoma, Malignant Fibrous ; Keloid ; Langerhans Cells ; Neurofibroma

Central odontogenic fibroma is a rare odontogenic neoplasm that originates from odontogenic ectomesenchyme. Here, a case of central odontogenic fibroma in a 17-year-old male is reported. Since the present case showed a multilocular radiolucency with partially ill-defined border between the right mandibular condyle and the distal root of the right mandibular third molar, differential diagnosis involved a wide range of pathosis from benign lesions like ameoloblastic fibroma and odontogenic myxoma to more aggressive lesions such as desmoplastic fibroma, juvenile aggressive fibromatosis, or fibrosarcoma.
Adolescent ; Diagnosis, Differential ; Fibroma ; Fibroma, Desmoplastic ; Fibromatosis, Aggressive ; Fibrosarcoma ; Humans ; Male ; Mandibular Condyle ; Molar, Third ; Myxoma ; Odontogenic Tumors