Ossifying Fibromyxoid Tumor: Invariable Ultrastructural Features and Diverse Immunophenotypic Expression

Hirose, Takanori; Shimada, Shio; Tani, Takao; Hasegawa, Tadashi

doi:10.1080/01913120701351157

Cited by 19 publications

(11 citation statements)

References 29 publications

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“…Although schwannian differentiation has generally been favored in OFMT, based on ultrastructural findings, such as the presence of well-developed, occasionally reduplicated external lamina, and S100 protein expression (8, 11, 16, 20), our gene expression data does not support this. Both by cluster analysis and principal component analysis OFMT were clearly distinct from nerve sheath myxoma and schwannoma, both highly differentiated schwannian neoplasms.…”

Section: Discussioncontrasting

confidence: 65%

“…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%

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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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Section: Discussioncontrasting

confidence: 65%

Section: Introductionmentioning

confidence: 93%

Ossifying Fibromyxoid Tumor of Soft Parts

Graham

Dry

et al. 2011

American Journal of Surgical Pathology

115

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“…Ossifying fibromyxoid tumor (OFMT), initially described by Enzinger et al in 1989, is an uncommon mesenchymal neoplasm that usually arises as a superficial mass in the subcutis. The histogenesis of OFMT has been a subject of much debate, with nerve sheath, myoepithelial, cartilaginous, and even osteogenic differentiation proposed, based on various immunohistochemical and ultrastructural studies . Recent genetic analyses indicate that over 80% have recurrent gene rearrangements, most commonly involving the PHF1 gene on 6p21 .…”

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confidence: 73%

Ossifying fibromyxoid tumor: a clinicopathologic analysis of 26 subcutaneous tumors with emphasis on differential diagnosis and prognostic factors

et al. 2015

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“…7 Uniquely, the tumor often demonstrates well-formed reduplicated basal lamina on electron microscopy which correlates immunohistochemically with positivity for collagen type IV. 7,9 The tumor's histogenesis is uncertain, but when first described it was suspected to be of cartilaginous or neural origin. 1 Since then studies have also suggested that it may be of Schwann cell, smooth cell, myopeithealial, skin adnexal, osseous, and chondroid origin, with Schwann cell origin being the most highly suspected type.…”

Section: Resultsmentioning

confidence: 99%

Malignant Ossifying Fibromyxoid Tumor of the Parapharyngeal Space

Tanna¹,

Chadha²,

Kori³

et al. 2009

The Laryngoscope

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Ossifying Fibromyxoid Tumor (OFMT) is a rare, recently described tumor. As such, there is a paucity of information in the literature regarding this neoplasm. Most reports cite that the tumor most commonly occurs subcutaneously in the soft tissues of the extremities. 1-3 Additionally, OFMT has a predilection for men and exhibits a moderate recurrence rate (27%). 2 Malignant forms of the tumor are even rarer than their benign counterparts. At the time of manuscript preparation, the authors found only 27 reported cases of the malignant OFMT (versus 82 reports of benign OFMT). Similar to benign OFMT, most of the reported malignant cases occurred subcutaneously in extremities. However, a few uncommon locations have been described such as the mandible, 4 oral cavity, 5 and scalp. 6 While rare occurrences in the head and neck have been noted, to the authors' knowledge no such tumor has been reported in the parapharyngeal space. The authors present a case of a malignant ossifying fibromyxoid tumor in the parapharyngeal space.A 33-year old Pakistani male presented for evaluation for a two-year history of progressively enlarging right-sided neck mass. The patient also complained of gradual dysphonia but denied any dysphagia, odynophagia, snoring, stridor, trismus, or otologic symptoms. His past medical and surgical history were otherwise non-contributory. He did report a history of nicotine use, having smoked _ pack per day for the past 16 years. He denied daily alcohol consumption.On physical examination, the patient had symmetric palatal elevation, but the tonsillar fossae were asymmetrical (right buldge). With palpation of the neck, a firm right neck mass was detected, located anterior and deep to the right sternocleidomastoid and spanned from the angle of the jaw to the midlevel of the neck. Flexible nasopharyngolaryngoscopy revealed a slight bulge of the right pharyngeal wall and partial obliteration of the right piriform sinus (Figure 1). Vocal cord abduction and adduction was otherwise intact and symmetric.Magnetic resonance imaging (MRI) of the lesion indicated a 5.2 x 5.0 x 7.0 right neck mass extending from the skull base to the level of the thyroid cartilage ( Figure 2). It showed areas of cystic degeneration and enhancement with gadolinium. Magnetic resonance angiogram revealed lateral displacement of the right internal jugular vein anterior displacement of the external and internal carotid arteries. There was however, no splaying of the external and internal carotid arteries. Preoperative FNA was not performed secondary to the potential risk of bleeding associated with a suspected paraganglioma.The patient underwent uncomplicated right transcervical excision of the parapharyngeal lesion. Histopathologic analysis of the mass demonstrated features consistent with malignant OFMT. It demonstrated high nuclear grade and cellularity along with >2 MF/ 50 HPF . Immunohistochemical studies showed the presence of basal lamina, immunoreactivity of the neopastic cells to S-100 protein and glial fibrillary acidic treatment...

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Ossifying Fibromyxoid Tumor: Invariable Ultrastructural Features and Diverse Immunophenotypic Expression

Cited by 19 publications

References 29 publications

Ossifying Fibromyxoid Tumor of Soft Parts

Ossifying Fibromyxoid Tumor of Soft Parts

Ossifying fibromyxoid tumor: a clinicopathologic analysis of 26 subcutaneous tumors with emphasis on differential diagnosis and prognostic factors

Malignant Ossifying Fibromyxoid Tumor of the Parapharyngeal Space

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