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

Zámečnı́k, Michal; Michal, Michal; Simpson, Roderick H.W.; Lamovec, Janez; Hlavčák, Peter; Kinkor, Z; Mukenšnábl, Petr; Matéjovský, Z; Betlach, J

doi:10.1016/s1092-9134(97)80011-1

Cited by 58 publications

(28 citation statements)

References 17 publications

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“…Evidence exists for a schwannian or neuronal differentiation but this has not been well proven. [18][19][20] Similarly, cartilaginous or myoepithelial differentiation was proposed by Enzinger et al 2 and Kilpatrick et al, 15 respectively. From these hypotheses, Graham et al 7 suggested a ''scrambled'' phenotype for these tumors.…”

Section: Differential Diagnosesmentioning

confidence: 84%

Ossifying Fibromyxoid Tumor: An Update

Bakiratharajan¹,

Rekhi

2016

Archives of Pathology &Amp; Laboratory Medicine

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Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain differentiation, initially described by Enzinger and colleagues. Until now, nearly 300 such cases have been reported worldwide. The histogenesis of these tumors remains controversial. These tumors show characteristic imaging findings and exhibit a spectrum of histopathologic features, including classical and atypical subtypes. Local recurrences and, occasionally, distant metastases have also been reported. A complete tumor resection forms the preferred treatment modality for these tumors, along with follow-up, as these tumors have an uncertain malignant potential. Lately, certain “molecular signatures” underlying OFMTs have been described that can further aid in reaching an accurate diagnosis for these tumors and unraveling their pathogenesis. This article is a review of the clinical, radiologic, histopathologic, and molecular features of OFMTs.

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Section: Differential Diagnosesmentioning

confidence: 84%

Ossifying Fibromyxoid Tumor: An Update

Bakiratharajan¹,

Rekhi

2016

Archives of Pathology &Amp; Laboratory Medicine

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“…Enzinger et al, in their original description, noted OFMT to have ultrastructural features suggestive of incomplete schwannian and/or cartilaginous differentiation (10). Subsequent immunohistochemical studies have also for the most part supported the concept of schwannian differentiation in OFMT (13, 16, 20, 29, 30, 39), although myoepithelial differentiation has also been suggested (18, 22). …”

Section: Introductionmentioning

confidence: 93%

“…In its classical form, OFMT is characterized by subcutaneous location, a peripheral shell of woven bone, and a vaguely lobular proliferation of small, bland round cells with very low mitotic activity, embedded in a fibromyxoid matrix (10). Although most OFMT conform to this morphologic description and show correspondingly benign clinical behavior, it has been recognized for some time that a subset of OFMT display atypical cytoarchitectural features, such as high cellularity or elevated mitotic activity, and show correspondingly more aggressive clinical behavior (13, 18, 39). Under the risk stratification system proposed in 2003 by Folpe and Weiss, OFMT are classified as “typical”, “atypical” or “malignant” based on their size, nuclear grade, cellularity, and mitotic activity (13).…”

Section: Introductionmentioning

confidence: 98%

Ossifying Fibromyxoid Tumor of Soft Parts

Graham

Dry

et al. 2011

American Journal of Surgical Pathology

115

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Ossifying fibromyxoid tumor of soft parts (OFMT) is a rare soft tissue and bone tumor of borderline malignancy displaying an uncertain line of differentiation. The existence of fully malignant OFMT is controversial. In order to better understand the natural history and line of differentiation taken by OFMT, we studied 46 cases by light microscopic, immunohistochemical (IHC), genomic, proteomic, and fluorescent in situ hybridization (FISH) methods. Cases were classified according to the 2003 Folpe and Weiss system. Clinical and follow-up information was obtained. IHC for S-100 protein, desmin, epithelial membrane antigen (EMA), cytokeratins, smooth muscle actin (SMA), INI-1, neurofilament protein (NFP), CD56d excitatory amino acid transporter-4 (EAAT4), and MUC4 was performed on formalin-fixed, paraffin-embedded (FFPE) tissues. Gene expression profiling and proteomic studies were performed on FFPE tissues from 13 and 5 cases, respectively. FISH for INI-1 was performed on 10 cases. The 46 tumors arose in 29 males and 17 females (median age 52 years, range 39-63 years) and involved the proximal (N=17) and distal extremities (N=13), head and neck (N=9) and trunk (N=5). Median tumor size was 5.4 cm (range 1.0-21.0 cm). Cases were classified as typical OFMT (26 of 46, 57%), atypical OFMT (5 of 46, 11%) and malignant OFMT (15 of 46 cases, 32%). Clinical follow-up (27 cases, median 55 months duration) showed all patients with typical and atypical OFMT to be alive without disease. Adverse events, including 3 local recurrences, 3 metastases, and 3 deaths, were seen only in malignant OFMT. IHC results were: S100 protein (30/41, 73%), desmin (15/39, 38%), cytokeratin (4/35 11%), EMA (5/32, 16%), SMA (2/34, 6%), INI-1 (lost in mosaic pattern in 14/19, 74%), EAAT4 (31/39, 80%), MUC4 (3/14, 21%), NFP (8/10, 80%) and CD56 (6/14, 43%). Gene expression profiling showed typical and malignant OFMT to cluster together, distinct from schwannian tumors. Proteomic study showed expression of various collagens, S100 protein, and neuron-related proteins. FISH showed INI-1 deletion in 5/7 (71%) cases. We conclude that malignant OFMT exist, and may be recognized by the previously proposed criteria of Folpe and Weiss. Expression of neuron-related, in addition to Schwann cell and cartilage-associated markers, suggests a “scrambled” phenotype in OFMT. Loss of INI-1 or other genes on 22q are likely important in the pathogenesis of these rare tumors.

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“…Este caso clínico muestra varias cosas, en primer lugar que en la literatura se han reportado aproximadamente 220 casos de TFO, principalmente a través de la comunicación de casos clínicos y algunas series de distintos tamaños [1][2][3][4][5][6][7][8][9] , destacando el estudio de Miettinen et al sados, tomando como base la muestra analizada por Enzinger y ampliando el período de análisis a 37 años. El TFO es un tumor de muy baja frecuencia, por lo que es fácil que sea confundido, como en este caso, en que fue interpretado como un cáncer anaplásico de tiroides, lo que previamente ya había sido reportado en la tiroides, pero con una neoplasia folicular 10 .…”

Section: Discussionunclassified

“…A pesar de esto, mantiene una característica que le otorga un mayor grado de agresividad que un TFO típico, que es la infiltración de estructuras vecinas, lo que no se explica por la radioterapia, además clínicamente la paciente evolucionó con disfagia progresiva, lo que habla de un tumor localmente agresivo. Se han descrito una serie de variantes atípicas de TFO que poseen un mayor grado de atipia celular y que clínicamente se manifiestan con mayor tendencia a recurrir localmente e incluso dar metástasis [3][4][5][6][7] , como en la serie de Folpe en que 8 pacientes, de un total de 70, tuvieron metástasis a distancia (principalmente pulmón). Así, la recurrencia variaría entre un 18% y un 22% según las distintas series, en plazos que van desde los 6 años hasta los 10 años, lo que concuerda con este caso.…”

Section: Discussionunclassified

Tumor fibromixoide osificante simulando un cáncer anaplásico de tiroides: Reporte de un caso

Amat

et al. 2009

Rev Chil Cir

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Ossifying fibromyxoid tumor of the neck. Case reportOssifying fibromyxoid tumor of soft parts in an uncommon tumor with a benign behavior but with a tendency to relapse. We report a 76 years old female presenting with a growing mass located in the anterior portion of the neck that was excised. The pathological study disclosed an anaplastic thyroid cancer. The patient received chemotherapy and was lost from follow up. Nine years later, she presented with progressive dysphagia. A neck CT scan showed a mass in the left thyroid base that completely included the cervical esophagus. The patient was operated and the mass was excised. The pathological diagnosis disclosed an ossifying fibromyxoid tumor.Key words: Ossifying fibromyxoid tumor, Anaplastic thyroid cancer. ResumenEl tumor fibromixoide osificante corresponde a una neoplasia infrecuente, de comportamiento benigno pero que presenta recurrencia en un tercio de los casos, comportándose como un sarcoma de bajo grado. Se reporta el caso de una paciente operada de tiroides en dos oportunidades en 9 años, cuyo diagnóstico final fue tumor fibromixoide osificante.Palabras clave: Tumor fibromixoide osificante, cáncer anaplásico de tiroides.

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

Cited by 58 publications

References 17 publications

Ossifying Fibromyxoid Tumor: An Update

Ossifying Fibromyxoid Tumor: An Update

Ossifying Fibromyxoid Tumor of Soft Parts

Tumor fibromixoide osificante simulando un cáncer anaplásico de tiroides: Reporte de un caso

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