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PubMed2006

Mediastinal and pulmonary metastases of malignant ossifying fibromyxoid tumor.

Ossifying fibromyxoid tumor is usually a benign tumor. However some of these tumors with histologic and clinical evidence of malignancy have also been reported and little information is available regarding surgery for metastatic ossifying fibromyxoid tumor. We present a case involving extensive excision of a huge metastatic ossifying fibromyxoid tumor occupying the upper mediastinum and upper half of the right hemithorax.

Adult↗

PubMed2006

Subcutaneous ossifying fibromyxoid tumor.

Ossifying fibromyxoid tumor (OFMT) is an uncommon neoplasm occurring predominantly in the deep soft tissues of the trunk and proximal extremities. The lineage of this rare tumor to date is still uncertain. We present a case of a subcutaneous OFMT that recurred 8 years after initial resection. The histological findings of the primary and recurrent lesions are compared along with the histologic features possibly associated with aggressiveness. Dermatopathologists should consider OFMT in the differential diagnosis of subcutaneous neoplasms with a myxoid matrix.

Diagnosis, Differential↗

PubMedDate not supplied

[Ossifying fibromyxoid tumor].

Ossifying fibromyxoid tumor (OFMT) is a rare tumor of unknown histogenesis. It consists of chains and trabecules of ovoid cells in the fibromyxoid stroma (ICD code 8842/0). Tumor is frequently surrounded in part by a capsule of laminar bone. It is more frequent in males 14-79 years of age, mean 44 years. Soft tissues of extremities is a preferable location, but body, neck, oral cavity, mediastinum and retroperitoneal tissue can also be the site of OFMT. More than 100 OFMT cases are reported abroad. It is frequently masked by other tumors of soft tissues. We describe 3 cases in our country: 2 in 65 and 66 year old females and in a 44 year old male. Lipoma and soft tissue sarcoma were suspected clinically. Fibroblasts were found cytologically before operation in one case. Diagnosis was established after total biopsy of the tumor. One of the females is under observation after the operation without recurrence during more than two years. Preoperative diagnosis is sometimes possible when characteristic osteoblastic foci are observed.

Adult↗

PubMed1998

Ossifying fibromyxoid tumor resembling parosteal osteosarcoma.

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm which varies in the amount of bone tissue laid down. We report on a case of OFMT in a 59-year-old male which mimicked a large parosteal osteosarcoma on radiography, MRI, and CT. T1-weighted MR images showed high-intensity areas which reflected fatty marrow. The metaplastic bone was connected to the cortex of the femur, but the tumor did not involve the medullary cavity. The tumor was irradiated and the patient was administered chemotherapy. Wide excision of the tumor and hip rotation plasty was performed. The patient has been disease free for 18 months.

Bone Neoplasms↗

PubMed1996

Ossifying fibromyxoid tumor of soft parts.

Ossifying fibromyxoid tumors (OFT) of soft parts are very rare neoplasms recently described by Enzinger et al. (1). They usually present as well-circumscribed, slow-growing, asymptomatic subcutaneous masses. A characteristic finding is a fibrous capsule and an incomplete peripheral shell of mature bone. Although most cases behave as benign tumors, a high rate of local recurrences have been described. We describe a 43-year-old male with a perianal subcutaneous tumor of 10 years evolution. The histopathology was compatible with OFT. Due to the subcutaneous location of OFT, both the dermatologist and dermatopathologist need to know about this rare tumor.

Adult↗

PubMed1996

Ossifying fibromyxoid tumor of soft parts: a case report and review.

Ossifying fibromyxoid tumor of soft parts is a recently described, rare but morphologically distinctive tumor of soft tissue, including subcutaneous soft tissue. We report a case of ossifying fibromyxoid tumor of soft parts which occurred in the subcutaneous tissue of a 68-year-old male, and review the clinical, histologic, and immunohistologic features of this tumor. Ossifying fibromyxoid tumor of soft parts tends to occur in the subcutaneous tissue and have been reported from the head and neck, upper and lower extremities, and trunk. Immunohistologic studies have supported a neural origin. While considered benign, local recurrence after excision is common and these tumors should be excised with clear margins to prevent recurrence.

Aged↗

PubMed1994

Ossifying subcutaneous tumor with myofibroblastic differentiation: a variant of ossifying fibromyxoid tumor of soft parts?

Immunohistochemical, ultrastructural, and flow cytometric studies were performed on an ossifying soft-tissue tumor, presumed to be a variant of ossifying fibromyxoid tumor of soft parts, which was located in the subcutis of the left buttock of a 76 year old Japanese woman. Histologically, this was a benign-looking spindle, oval, or round cell lesion, having a fibrous capsule with a discontinuous rim of bone as seen in typical cases. However, this lesion was also characterized by a high degree of cellular proliferation in storiform and whorl arrangements, extensive ossification, osteoid and metaplastic bone formation and absence of myxoid features. In an immunohistochemical study using formalin-fixed, paraffin-embedded sections, many tumor cells expressed vimentin, S-100 protein, Leu-7, neuron specific enolase, and desmin. Ultrastructurally, this neoplasm consisted of fibroblast-like cells and myofibroblast-like cells. This tumor had an aneuploid DNA content. No recurrence has been observed for 16 months. These results suggest that the neoplastic cells may show the phenotypic expressions of myofibroblast and also osteogenic differentiation.

Aged↗

PubMed1997

[A case of ossifying fibromyxoid tumor].

Recently described entity: ossifying fibromyxoid tumour of soft parts is presented. Schwann cell nature of the proliferating cells was demonstrated by immunohistochemical means. This special kind of nerve sheath tumors is characterised by pathological differentiation of the cells, resulting bone and in some cases additional cartilage formation. As for biological behaviour: in spite of occasional histological evidences of malignancy, no distant metastases are to be expected. Local aggressivity and recurrences may occur, thus designation is preferable as semimalignant or according to more modern terms as low-grade malignant tumour.

Adult↗

PubMed2002

Ossifying fibromyxoid tumor of soft parts. Cytogenetic findings.

Ossifying fibromyxoid tumor (OFMT) of soft parts is a recently described, rare but morphologically distinctive soft tissue tumor. The histogenesis of this lesion remains uncertain, although several immunohistochemical and ultrastructural features suggest that it is an unusual neural tumor, possibly of Schwann cell origin. We report here a case of a malignant variant of OFMT that occurred in the foot of a 52-year-old man. The karyotype of a pulmonary metastasis exhibited the following complex numeric and structural aberrations:72 approximately 74,XXY,-5,+6,+del(8)(p21),del(9)(p22),+10,der(11)t(3;11)(p21;p15),del(12) (q13),der(13)t(5;13)(q13;q34),+18,+19,+20,-22 [cp10]. A kidney metastasis exhibited the following karyotypic abnormalities: 46,XY,add(3)(p11),+der(3)t(3;?;11)(3qter-->3p11::?::11q13-->11qter), -5,del(8)(p21),add(9)(q22),del(9)(p22),der(11)t(3;11)(p21;p15),del(12)(q13),+der(13)t(5;13) (q13;q34),-22. To our knowledge, this is the first reported case of OFMT in which clonal chromosomal aberrations have been shown.

Chromosome Aberrations↗

PubMed1995

Atypical and malignant variants of ossifying fibromyxoid tumor. Clinicopathologic analysis of six cases.

Ossifying fibromyxoid tumor (OFMT) of soft parts is a recently defined fibro-osseous neoplasm, the biologic behavior of which is generally regarded as benign. We report six variant cases of OFMT with histologic features of malignancy, two of which behaved aggressively. All these tumors arose in the extremities of adults (aged 36-76 years), and five of the six were subcutaneous. Four patients were men. Macroscopically, all the tumors were well circumscribed and somewhat lobulated. Cardinal morphologic features included lobules of round to spindled cells within a fibromyxoid matrix and randomly distributed, often centrally located osteoid within which were plump neoplastic cells. In contrast to typical OFMT, a hypocellular, cytologically benign, lamellar bony shell was observed only focally; cellularity was increased (four cases), and mitotic activity was frequent, exceeding two mitotic figures per 10 high-power fields (three cases). One case associated with metastases was morphologically bland. Immunohistochemically, positivity for S-100 protein was observed in the primary tumors of three cases and in the pulmonary metastasis of a fourth. Desmin was positive in one case. Ultrastructural features in three cases were very similar to usual OFMT. Clinical follow-up revealed local recurrence in two cases; one patient has developed recurrent pulmonary metastases. We believe these findings support the view that some atypical cases of OFMT exhibit morphologic patterns that might predict more aggressive behavior.

Adult↗

PubMed2005

Ossifying fibromyxoid tumor of the skin: a report of 2 cases with light microscopic, immunohistochemical, and electron microscopic characterization.

Ossifying fibromyxoid tumor is a rare fibro-osseous neoplasm occurring predominantly in the subcutaneous tissue and/or skeletal muscle of the extremities. The immunohistochemical and ultrastructural studies in reported cases favor neural or Schwannian origin. To date, ossifying fibromyxoid tumor presenting as a primary skin tumor has not been described in the literature. Two cases of primary cutaneous ossifying fibromyxoid tumor with light microscopic, immunohistochemical, and ultrastructural characterization are presented. Our observations also support neural differentiation of these tumors.

Female↗

PubMed1996

Ossifying fibromyxoid tumor of the retroperitoneum.

A case of ossifying fibromyxoid tumor (OFMT) in an unusual site, the retroperitoneum, in a 71 year old Japanese man is reported. The well demarcated tumor contained a large amount of mature bony tissue within the tumor. The tumor cells were round to spindle-shaped, and showed a high cellularity in some areas and were loosely arranged in other areas. Although the majority of tumor cells expressed vimentin, neuron specific enolase and Leu-7, there were no tumor cells positive for S-100 protein, myogenic or epithelial markers. We would like to emphasize that tumors classified as OFMT contain a wide spectrum of histologic features and heterogeneity.

Aged↗

PubMed2004

Cytologic diagnosis of ossifying fibromyxoid tumor of soft tissue: a case report.

Ossifying fibromyxoid tumor (OFMT) of soft tissue is an uncommon, recently described neoplasm. It is usually, but not universally, a benign neoplasm and cases of metastatic so-called "malignant OFMT" have been reported. Although the histopathologic features of OFMT are well characterized, the same is not entirely true so far as the cytology literature is concerned. There are only three cytologic descriptions of OFMT to date to the best of our knowledge. A 62-year-old woman presented with a subcutaneous well-circumscribed nodular soft tissue mass at the upper end of the shoulder. X-ray and computed tomography revealed an incomplete shell of calcification surrounding the nodular mass along with foci of ossification within it. Fine-needle aspiration (FNA) cytology showed round to polygonal to spindle shaped neoplastic cells arranged in clusters, cords, and small aggregates and lying discretely in a mucoid background. Some osteoid-like material was also seen. The tumor was completely excised and histopathologic examination confirmed the diagnosis of OFMT. OFMT should be considered as a possibility in FNA cytology of myxoid soft tissue tumor especially with radiologic evidence of ossification.

Biopsy, Fine-Needle↗

PubMed1993

Ossifying fibromyxoid tumor of soft parts: a case report.

A case of ossifying fibromyxoid tumor of soft tissue parts in the presacral region, which developed in a 46-year-old Japanese man is reported. The enucleated tumor was an encapsulated, elastic, hard mass measuring 2.5 x 2.2 x 1.4 cm. Microscopically, the tumor was characterized by foci of round to polygonal cells dispersed in a fibromyxoid matrix, containing varying amounts of collagen and osteoid tissue. At the marginal zone, the shell of bone and osteoid tissue with calcification separated the tumor from the surrounding tissues. Our patient is showing no evidence of recurrence after a one-year follow-up period. The findings are compatible with those proposed for ossifying fibromyxoid tumor of soft parts by Enzinger et al. in 1989. This tumor should be differentiated from cartilaginous, osteogenic and neural tumors.

Fibroma↗

PubMed2006

Resection of metastatic pulmonary lesion of ossifying fibromyxoid tumor extending into the left atrium and ventricle via pulmonary vein.

The ossifying fibromyxoid tumor (OFMT) of soft parts is a rare soft tissue neoplasm of uncertain lineage. The most common metastases are found in the lung. Herein, we present the first case report of pulmonary metastasis of ossifying fibromyxoid tumor with intracardiac extension, which was resected carefully using cardiopulmonary bypass and cardiac arrest. Subsequently pulmonary left lower lobectomy was performed. The patient recovered uneventfully and was discharged in after a few days. Recently, OFMT has been considered as a tumor of intermediate malignancy. We recommend wide surgical excision of primary tumor and radiotherapy. Early follow-up is mandatory.

Aged↗

PubMedDate not supplied

Fine needle aspiration cytology of the malignant variant of ossifying fibromyxoid tumor of soft parts: a case report.

BACKGROUND: Ossifying fibromyxoid tumor (OFMT) of soft parts is a rare, recently defined, fibroosseous neoplasm, generally regarded as clinically benign; however, one-third of cases recur locally, and several malignant examples have been reported. Fine needle aspiration (FNA) cytology of the tumor is rarely described in the literature. We provide the first cytomorphologic study of the malignant variant. CASE: A 70-year-old man presented with an intramuscular mass in the right buttock. Computed tomography revealed ossification within the mass and multiple pulmonary nodules. FNA biopsy showed round and polygonal to spindled tumor cells, arrayed singly, cordlike or in small aggregates, with scattered dense stromal fragments and a slightly myxoid background. The nuclei showed significant pleomorphism accompanied by coarse chromatin with clumping, irregular contours, and one to two distinct nucleoli. The tumor cells were recognizable as sarcoma, with no evidence of high grade malignancy. The tumor was totally excised, histopathologically confirmed as the malignant variant of OFMT of soft parts, and immunohistochemically and ultrastracturally analyzed as of neural origin. CONCLUSION: The FNA specimen revealed that the cytomorphology was consistent with the histologic features of the malignant variant of OFMT, but several characteristic histologic parameters, such as multilobular proliferation and peripherally placed mature, bony trabeculae, were not reflected in the aspirates. Although FNA cytologic findings may be of limited diagnostic utility in OFMT, radiographic evidence of calcification/ossification suggests that OFMT should be subjected to differential diagnosis with fine needle aspiration biopsy of soft tissue tumors. Additional studies will be required for further clarification.

Aged↗

PubMedDate not supplied

Ossifying fibromyxoid tumor of the orbit.

PURPOSE: This report describes the clinical, radiographic, and histopathologic features of the first known case of ossifying fibromyxoid tumor involving the orbit. METHODS: Case report and literature review. RESULTS: An 81-year-old woman presented with a 3-month history of diplopia, pain, and right upper eyelid swelling. CT and MRI of the orbit disclosed a 2.5-cm extra-conal mass superomedially in the right orbit, with displacement of the globe inferiorly and remodeling of the orbital roof. During surgery, the tumor was noted to be firm, gritty, and extra-periosteal in location, and the bone adjacent to the tumor was irregular and abnormal in consistency. The tumor was removed intact without difficulty. Histologic, immunohistochemical, and transmission electron microscopic examination disclosed features consistent with ossifying fibromyxoid tumor. Fourteen months after removal, the tumor recurred and the patient underwent a second excisional biopsy. The tumor recurred a second time 30 months after the first surgery. The patient underwent a third surgery to remove the tumor and received adjuvant radiation therapy. She has been free of disease for more than 18 months. CONCLUSIONS: This is the first known case of ossifying fibromyxoid tumor involving the orbit. Increased cellularity, nuclear atypia, and mitotic activity have been associated with metastatic and recurrent potential. The tumor in this case had moderate cellularity, low nuclear grade, and no mitotic figures. However, the close proximity of the tumor to important structures within the orbit precluded its excision with wide margins and may have predisposed the patient to the two episodes of local recurrence.

Aged, 80 and over↗

PubMed1997

Ossifying fibromyxoid tumor of soft parts: a report of 17 cases with emphasis on unusual histological features.

Ossifying fibromyxoid tumor of soft parts is an unusual benign neoplasm, with a tendency for local recurrence. Its typical microscopic appearance is that of a multinodular proliferation of round to spindle shaped cells separated by fibrous bands in which bone formation is often seen. Herein, we present the clinicopathologic features of 17 examples of this tumor with particular emphasis on some unusual histopathologic features that may place pitfalls in the diagnosis of this tumor, including satellite micronodules, mucinous microcysts, absence of myxoid areas, crush artifact, multiple microcalcifications, epidermoid cysts, atypical chondroid differentiation with binucleate lacunar cells, pericytic growth pattern, and malignant change. Awareness of these unusual morphologic features should lead to a search for areas displaying the more typical features of ossifying fibromyxoid tumor to arrive at a correct diagnosis.

Adolescent↗