Neurothekeoma Research Articles

Recurrent Neurothekeoma of the Foot

Nerve sheath myxomas (NEUROTHEKEOMA) are uncommon tumors of nerve sheet origin. These tumors most frequently arise from in the dermis and subcutaneous tissues of the head and neck, and upper extremities. Neurothekeoma may be either asymptomatic or may present as a painful raised, skin colored, well circumscribed dermal lesion averaging 1 cm in diameter. This entity is especially rare in the foot and we present a 15 years old male patient with a large (6.5 cm) recurrent eurothekeoma of the left foot.

Neurothekeoma in the Posterior Fossa: Case Report and Literature Review

Neurothekeoma is a benign nerve sheath tumor, also known as nerve sheath myxoma. It arises from the cutaneous nerves of the head and neck region. In certain cases, neurothekeoma has been reported in the breast, oral cavity, tongue, maxilla, and spinal intradural space. Intracranial neurothekeoma, however, is an extremely rare entity, with only three cases reported in the literature: one in the parasellar region, one in the deep white matter, and another one in the cerebellopontine angle. We present the case of a 40-year-old man with a very large neurothekeoma present in the posterior fossa who had no neurologic deficit on presentation.

P63 Immunohistochemical Staining Is Limited in Soft Tissue Tumors

p63 is a p53 homolog that is expressed in various normal epithelial tissues and epithelial malignancies. Its expression in mesenchymal lesions has not been examined in depth; therefore, we studied p63 expression by immunohistochemical analysis in 650 soft tissue tumors. We found that p63 expression is limited in soft tissue tumors. The majority of tumors studied were p63-, including all cases of angiosarcoma, lipomatous neoplasms, dermatofibrosarcoma protuberans, solitary fibrous tumor, schwannoma, neurofibroma, gastrointestinal stromal tumor, and leiomyosarcoma. Nuclear p63 reactivity was found in a subset of soft tissue myoepithelioma and myoepithelial carcinoma of soft tissue, cellular neurothekeoma, soft tissue perineurioma, Ewing sarcoma/peripheral neuroectodermal tumor, diffuse-type giant cell tumor, and giant cell tumor of soft parts. Infrequent, weak, or focal p63-staining patterns were observed in low-grade fibromyxoid sarcoma, malignant peripheral nerve sheath tumor, extraskeletal myxoid chondrosarcoma, myxofibrosarcoma, proximal-type epithelioid sarcoma, synovial sarcoma, embryonal rhabdomyosarcoma, desmoplastic small round cell tumor, atypical fibroxanthoma, and spindle cell melanoma. Absent p63 expression is typical for most soft tissue tumors, including most (but not all) that would be in the differential diagnosis of spindle cell squamous carcinoma.

Agminated cellular neurothekeoma

Cellular neurothekeoma represents a benign, slow-growing neoplasm that typically occurs as a solitary lesion on the face, neck or arm. Reports of multiple lesions are rare. To our knowledge, multiple lesions occurring as eruptive clusters localized to a single anatomical site has not been previously reported. This report details a case of an agminated cellular neurothekeoma occurring on the nose of a 28-year-old man. Recognition of multiple localized eruptive lesions of cellular neurothekeoma is important in order to facilitate correct diagnosis and avoid unnecessary treatment.

Orbital Nerve Sheath Myxoma: A Case Report

A 72-year-old man was referred to the Service of Ophthalmology due to a 2-year history of ptosis and a painless mass in the lateral orbital margin of the right eye. Orbital MRI revealed a well-demarcated lesion in the superotemporal quadrant of the orbit. After surgical excision, histopathological examination led to the diagnosis of nerve sheath myxoma, a tumor composed of myxoid nodules separated by fibrous septa with spindle-shaped and stellate cells. Many of these cells were immunostained with antibodies to S-100 protein. This is the first case reported in the literature of such a tumor located in the orbit, and, though extremely rare, it should be considered in the differential diagnosis of orbital tumors.

Un sarcome cutané congénital rare : la tumeur fibrohistiocytaire plexiforme

We report the unusual case of a three-year-old girl which presented since birth a pigmented tumor of the left side 0.5cm in diameter. Surgical removal was decided given the hypothesis of a congenital naevo-cellular naevus. The histological study ended with the diagnosis of plexiform fibrohistiocytic tumor (PFHT). Two other congenital PFHT have been reported until now. PFHT is a rare mesenchymal neoplasm of intermediate malignancy, first reported by Enzinger and Zhang in 1988 which may be difficult to diagnose, because of its low frequency, particulary in congenital cases. It is important to distinguish it from others childhood cutaneous tumors (particulary plexiform neurofibroma, cellular neurothekeoma, infantile myofibromatosis, and fibrous hamartoma). The tumor has a high local recurrence rate and complete surgical resection of the tumor, with wider margins, is required.

Cellular Neurothekeoma With Fascicular Growth Features Mimicking Cellular Dermatofibroma

Cellular neurothekeoma (CNT) is a benign cutaneous mesenchymal neoplasm. Most demonstrate a lobulated to micronodular architecture. Rarely, CNT demonstrates a predominantly fascicular growth pattern, without prominent sclerosis and thus can mimic cellular dermatofibroma (DF). Three CNT with a predominantly fascicular pattern were obtained. The clinicopathologic features and accompanying immunohistochemical stains were evaluated. All cases demonstrated a moderately cellular proliferation of epithelioid to spindle cells with pale to eosinophilic slightly granular cytoplasm, vesicular nuclei, and a single nucleolus arranged in a fascicular pattern with thick collagen bundles at the periphery (collagen trapping). One case had prominent epidermal hyperplasia. The neoplastic cells expressed NKI-C3, CD10, and micropthalmia transcription factor and lacked expression of factor XIIIa, S-100, epithelial membrane antigen, and CD34. Our cases showed an unusual pattern of CNT with a predominantly fascicular growth pattern, thickened collagen bundles at the periphery, and occasionally epidermal hyperplasia. The overlap with cellular DF is striking. The presence of plump to epithelioid cytomorphology with abundant cytoplasm, with focally prominent nucleoli; the presence of focal lobulated to micronodular growth pattern along with micropthalmia transcription factor positivity; and lack of factor XIIIa expression are helpful in distinguishing fascicular CNT from cellular DFs.

Cellular neurothekeoma with neuroendocrine differentiation

We report a case of cellular neurothekeoma with unusual clinicopathological features in which neuroendocrine markers, determined by immunohistochemistry were observed. Histologically, the tumor showed a micronodular architecture with hypercellular lobules composed of slightly spindled to epithelioid cells, with nuclear atypia or pleomorphism and extension into fat, skeletal muscle. Neoplastic cells were immunoreactive for NKI/C3, CD68, CD10, and smooth-muscle actin, whereas S100 and HMB-45 staining was negative. An intriguing feature was the strong expression by tumor cells of different neuroendocrine markers. Clinical follow up showed no local recurrences after five months despite the presence of positive margins. The presence of atypical histopathological features may cause diagnostic problems with malignant mesenchymal tumors, nevo-melanocytic lesions, and fibrohistiocytic tumors. The immunohistochemical profile including the positive staining for neuroendocrine markers may suggest divergent differentiation or an origin from myofibroblast and neuroendocrine cells.

Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis

Neurothekeomas and dermal nerve sheath myxomas have previously been considered related cutaneous neoplasms of peripheral nerve sheath origin based on light microscopic similarities. However, recent immunohistochemical and ultrastructural data indicate nerve sheath myxomas exhibit true nerve sheath differentiation, whereas no such compelling evidence exists for neurothekeomas. Although neurothekeomas lack a specific immunohistochemical profile, similar antigen expression and histopathologic patterns suggest neurothekeomas may be categorized as fibrohistiocytic tumors. To date, no known molecular studies have examined the histogenetic relationship of these tumors. We report the first microarray-based gene expression profile study of these entities on formalin-fixed paraffin-embedded tissues. Cases of dermal schwannomas, dermal nerve sheath myxomas, myxoid/mixed/cellular neurothekeomas, and cellular fibrous histiocytomas diagnosed in the past 3 years were identified in our database. Archival formalin-fixed paraffin-embedded tissue from 28 patients was selected for microarray analysis (seven schwannomas, five nerve sheath myxomas, nine myxoid/mixed/cellular neurothekeomas and seven cellular fibrous histiocytomas). Following tumor RNA isolation, amplification and labeling using commercially available kits, labeled targets were hybridized to the Affymetrix GeneChip Human Genome U133 Plus 2.0 Array (Santa Clara, CA, USA). Acquisition of array images and data analyses was performed using appropriate software. Hierarchical clustering and principal component analysis demonstrated discrete groups, which correlated with histopathologically identified diagnoses. Dermal nerve sheath myxomas demonstrate very similar molecular genetic signatures to dermal schwannomas, whereas neurothekeomas of all subtypes more closely resemble cellular fibrous histiocytomas. We are the first to report distinct gene expression profiles for nerve sheath myxomas and neurothekeomas, which further substantiates the argument that these are separate entities. Our molecular data confirms that dermal nerve sheath myxomas are of peripheral nerve sheath origin, and suggests that neurothekeomas may actually be a variant of fibrous histiocytomas.

Mohs micrographic surgery for the treatment of cellular neurothekeoma and review of its use in surgical management of benign tumors

Mohs micrographic surgery for the treatment of cellular neurothekeoma and review of its use in surgical management of benign tumors

Nerve sheath myxoma (neurothekeoma) of cerebellopontine angle: case report of a rare tumor with brief review of literature.

Nerve sheath myxoma (neurothekeoma) are rare benign nerve sheath tumors, usually arising in the skin of the head and neck region and upper extremities in young females. To the best of author's knowledge only two cases of intracranial neurothekeoma have been published in the English literature. These tumors were located in the parasellar area and in middle cranial fossa. This is the first case report of cerebellopontine angle neurothekeoma and third case report of intracranial neurothekeoma. This patient, a 45-year-old female, presented to us with complaints of right side progressive hearing loss for 12 months and swaying during walking for 8 months. Histologically the tumor had lobular appearance with spindle or stellate cells embedded in abundant myxoid background. The tumor cells were diffusely positive for S100. The patient was symptom free at eight month follow up.

Cellular Neurothekeomas Arising on a Café-au-Lait Macule

Neurothekeoma is a tumor probably derived from the nerve sheath, which usually appears during the first 2 decades of life as a solitary papule or nodule; multiple neurothekeomas are very unusual. We present a case of multiple cellular neurothekeomas arising on a café-au-lait macule in a child, a situation that has not been reported to our knowledge.

Multiple Desmoplastic Cellular Neurothekeomas Localized to the Face of a 16-Year-Old Boy

Cellular neurothekeomas are relative uncommon benign dermal tumors of uncertain histogenesis. Most commonly they arise as a solitary papule or nodule on the head and neck or upper trunk of young adults with a slight female predominance. There has been only 1 previous report of multiple neurothekeomas. The patient described herein was a 16-year-old otherwise healthy boy who presented with approximately 30 facial papules that arose over the course of 6 months and progressively enlarged. Histologically, all lesions were composed of spindled to epithelioid cells, but varied in the degree of cellularity and dermal sclerosis. Immunohistochemical staining showed that the cells of interest expressed S100A6, vimentin, CD63 (NKI/C3), PGP 9.5, and factor XIIIa and were negative for CD68, glial fibrillary acid protein (GFAP), S-100, HMB-45, epithelial membrane antigen, actin, and CD57 consistent with a diagnosis of multiple desmoplastic cellular neurothekeomas.

Fibrohistiocytic Tumors Containing Zebra Body-like Inclusions and Fibripositors

Plexiform fibrohistiocytic tumor (PFH) and cellular neurothekeoma (CN) are dermal neoplasms that present many histological and immunophenotype similarities as well as unknown histogenesis. Recently, the ultrastructural detection of zebra body‐like inclusions in lesional histiocytes has been considered crucial to favor the diagnosis of PFH over that of CN. Here we report two examples of dermal tumors that were diagnosed as CN. Electron microscopy revealed cytoplasmic collections of myelin and zebra body‐like inclusions in tumor cells; these inclusions focally merged together or with multivesicular bodies; tumor cells also showed collagen secretion granules and fibripositors, i.e., channels containing single, double or multiple copies of collagen fibrils. These observations support the view that PFH and CN have a common histogenesis and consists of cells sharing phagocityc and fibrillogenic abilities.

Classic neurothekeoma (nerve sheath myxoma) and cellular neurothekeoma of the oral mucosa: immunohistochemical profiles

Classic neurothekeoma (nerve sheath myxoma) is regarded as being a true benign cutaneous tumor of nerve sheath origin. Cellular neurothekeoma was separated from the classic type by histogenesis, morphology and immunophenotype. Whether cellular neurothekeoma represents a continuum within the spectrum of classic neurothekeoma or is an independent entity is controversial. Only a small number of classic neurothekeomas of the oral mucosa have been reported and there are even fewer publications on cellular neurothekeoma. We analyzed a series of oral neurothekeomas (classic and cellular) with a panel of neural and other mesenchymal markers to enhance their diagnosis and classification. One cellular and three classic neurothekeomas were submitted to a panel of immunohistochemical stains with antibodies against S100, S100A6, NSE, NKI/C3, PGP9.5, α-SMA, HHF-35, CD68 and vimentin. Two cases of neurofibroma (plexiform type), representing a true lesion of neural origin, served as control. The cellular neurothekeoma yielded a positive immunoreaction for S100A6 and NKI/C3 and a negative immunoreaction for S-100. The classic neurothekeomas demonstrated a positive reaction for S-100 and S100A6, but a negative one for NKI/C3. Other markers were non-contributory to distinguishing between these types of lesions. The small number of reported oral neurothekeomas (classic and cellular) could be due, in part, to the lack of recognition of their particular morphologic and immunohistochemical features. Our results indicate that testing for NKI/C3 immunoreactivity may be of value in distinguishing between cellular and classic neurothekeoma.

Neurothekeoma Palpebrae: A Report of 3 Cases

Neurothekeoma palpebrae is the diagnostic term used to describe nerve sheath myxoma or neurothekeoma of the eyelid. Although these tumors are not uncommonly found in the head and neck region, eyelid involvement is very uncommon. We present 2 cases of cellular neurothekeoma and 1 case of nerve sheath myxoma occurring in the eyelid. Patient 1 was a 13-year-old girl with a left upper eyelid lesion of 6-months duration. Microscopic examination disclosed a cellular neurothekeoma with mild myxoid change and osteoclast-like multinucleated cells. Tumor cells infiltrated the orbicularis oculi muscle. The tumor cells were immunopositive for NKI.C3, CD34, and focally for S-100 protein. Multinucleated cells were reactive to CD68. Mart-1, smooth muscle actin, CD31, keratin, desmin, myogenin, synaptophysin, and neurofilament protein (NFP) were negative. Patient 2 was a 4-year-old girl with a left upper eyelid lesion diagnosed clinically as a chalazion. The lesion was incised. Five months later, the patient returned with a firm 5.5-mm nodule at the site. Excision revealed cellular neurothekeoma invading the orbicularis oculi. Tumor cells were NKI.C3 immunopositive and S-100 protein negative, and the multinucleated cells were CD68 positive. Patient 3 was a 70-year-old woman with a 10-year history of a subcutaneous right lower eyelid nodule which had recently changed color. The excised specimen demonstrated a hypocellular, well-circumscribed myxoid tumor surrounded by a thin rim of fibrous connective tissue. Tumor cells contained moderate amounts of eosinophilic cytoplasm with irregular, hyperchromatic nuclei. Nucleoli and mitotic figures were not apparent. Tumor cells were NKI.C3 negative, and S100 protein and glial acidic fibrillary protein positive, consistent with a nerve sheath myxoma. Including our 3 cases, 10 cases of nerve sheath myxoma and neurothekeoma occurring in the eyelid have been reported the English language literature. Although uncommon, these lesions should be kept in the differential diagnosis of eyelid tumors that can masquerade as chalazia.

Mohs Micrographic Surgery for the Treatment of Cellular Neurothekeoma and Review of Its Use in Surgical Management of Benign Tumors

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Nerve Sheath Myxoma (Neurothekeoma) of the Gingiva, A Case Report and Review of the Literature

Nerve sheath myxoma (NSM) is a benign peripheral nerve sheath tumor that rarely occurs in the oral cavity. Among the 23 reported intraoral cases, no lesion has previously been reported on the gingiva. In this report, we describe the first gingival case of oral neurothekeoma with histopathologic and immunohistochemical characteristics. The patient, a 32 year old female presented with a slowly growing gingival mass diagnosed clinically as an epulis. The lesion was surgically excised. Histopathologically, the lesion presented as a submucosal multinodular mass composed of spindle and stellate-shaped cells with a myxoid background. Immunohistochemically, the tumor cells were sporadically positive for S-100 and NSE and negative for GFAP, EMA, SMA, CD68 and HMB45. The immunoprofile of this lesion confirmed a Schwann cell origin. The lesion was followed up for 10 months with no reports of recurrence.

이개에서 발생한 점액양 신경초점액종 1예

Neurothekeoma is a benign soft tissue tumor commonly located on the skin. According to the histopathologic features and expression of S-100 protein, it is subdivided into myxoid or cellular neurothekeoma. Here we report a rare case of the patient who presented with an isolated soft-tissue tumor in the posterior aspect of the auricle, which was diagnosed as myxoid neurothekeoma with positive S-100 protein. Preoperative radiologic feature of this tumor is also discussed in this case. To our knowledge, this is the first case of myxoid neurothekeoma arising from the auricle and confirmed with immunohistochemical stain of S-100 protein. (J Clinical Otolaryngol 2010;21:71–73)

Characterization of an intracranial neurothekeoma: case report

Neurothekeomas are benign tumors of presumed neural sheath origin. They are primarily found in superficial soft tissues, located in the upper portion of the body. Here, we report a case of intracranial neurothekeoma. A 37-year-old female presented at our clinic with sudden-onset left hemifacial pain of varying duration. The physical and neurological examination findings were normal. The magnetic resonance imaging scan showed a mass compressing the pons and extending from the medial section of the left middle fossa to the posterior fossa. The patient was operated on using a left presigmoid transpetrosal approach and the mass was totally removed. Neurothekeomas, also known as nerve sheath myxomas, are rare benign tumors. There have been two previous cases reported with an intracranial location. The information presented here now represents the third such case in the literature.