Nerve sheath myxoma

We studied telocytes/CD34+ stromal cells in the normal and pathological peripheral nervous system (PNS), for which we reviewed the literature and contributed our observations under light and electron microscopy in this field. We consider the following aspects: (A) general characteristics of telocytes and the terminology used for these cells (e.g., endoneurial stromal cells) in PNS; (B) the presence, characteristics and arrangement of telocytes in the normal PNS, including (i) nerve epi-perineurium and endoneurium (e...

Soft-tissue tumors are rare in the pediatric population. First described in 1969 as myxoma of the nerve sheath, the neurothekeoma is a benign tumor lesion with presumable origin in the nerve sheath. It occurs mainly in female children and presents as a mass of slow, subcutaneous growth, asymptomatic and without alteration of the local pigmentation. It is predominantly located in the head, neck, and extremities of the upper limbs. This report presents the case of a 16-year-old male with a tumor mass originating from the nerve sheath in the 4 th left metacarpal, intraosseous, and relapsed after previous surgical resection 2 years before this observation...

OBJECTIVE: Benign peripheral nerve sheath tumors (PNSTs) include mainly schwannomas-the most common tumors arising from peripheral nerves-and neurofibromas. Due to their origin, distinguishing between functional intact nerve and the fibers from whence the PNST arose may not always be easy to perform. The introduction of intraoperative tools to better visualize these tumors could be helpful in achieving a gross-total resection. In this study, the authors present a series of patients harboring PNST in which the surgery was performed under fluorescein guidance...

Neurothekeomas, or simply nerve sheath myxomas, are rare benign skin neoplasms. They are believed to derive from peripheral nerve sheath transformation and are typically more prevalent in females. They tend to be found mainly on the upper limbs as well as head and neck locations. In this report, we detail two children with neurothekeomas, describe the fundamental clinical manifestations, and review the current literature.

BACKGROUND: Oral neural tumors (ONTs) are rare lesions and represent reactive or neoplastic proliferations of nerve sheath cells. The purpose of the present study is to report the clinical, demographic and histopathologic features of 157 ONTs diagnosed in a single Oral Pathology Department and review the pertinent literature. MATERIAL AND METHODS: 157 cases of ONTs diagnosed during a 44-year period were retrospectively collected and the diagnosis was reconfirmed by studying representative haematoxylin and eosin stained tissue sections...

Intramuscular myxomas are rare, benign mesenchymal tumors. Tumor location in the forearm is very rare among all the intramuscular myxomas. To the best of our knowledge, there were two cases of supinator intramuscular myxoma reported in the literature, and we intend to report the third case of supinator myxoma with encasement of the posterior interosseous nerve (PIN). A 67-year-old lady presented with history of left proximal forearm swelling for the past 5 years. Magnetic resonance imaging showed a lobulated multiseptated lesion seen within left supinator muscle, which was hyperintense on T2-weighted images (T2WI) and hypointense on T1-weighted images (T1WI), with peripheral enhancement post contrast...

Multinodular accumulation of stromal mucin in breast known as nodular mucinosis is an extremely rare condition of breast. Alternatively it is known as myxoma or nerve sheath myxoma of breast. This benign lesion is confined to only nipple and areola region presenting as slow growing, soft and non tender lobulated mass. Histopathologically it reveals well circumscribed non-encapsulated myxoid/mucinous lesion with few infiltration of spindle cells within a collaginized stroma. The mucinous substance is stained positively with Alcian blue...

Melanotic malignant peripheral nerve sheath tumor is an extremely rare tumor, which originates from the neural crest, and more than half the cases are associated with Carney complex (myxomas, spotty pigmentation, and endocrine abnormalities). Herein, we have presented a case of a melanotic malignant peripheral nerve sheath tumor, which is not associated with Carney complex. The patient underwent preoperative nonenhanced CT, contrast-enhanced CT, and F-FDG PET/CT scans, which showed a large pelvic tumor with heterogeneous enhancement and increased F-FDG uptake...

Nerve sheath myxoma, a superficial myxoid tumor, was first described in 1969 by Harkin and Reed. Tumor has cytological and histological resemblance with neurothekeoma, another cutaneous myxoid lesion. Nerve sheath myxoma affects individuals of all age groups and equal predilection for both genders with most favored sites being the fingers and knee. Here, we present a case report of nerve sheath myxoma of the scalp, diagnosed and confirmed with histopathology and immunohistochemistry.

BACKGROUND: Neurothekeomas are slow-growing, well-circumscribed benign neoplasms. They usually involve cutaneous or subcutaneous tissues. Although originally described as myxomas deriving from nerve sheath cells, their exact histological classification is still uncertain. Peripheral nerve localization is rarely reported. Here, we describe a unique case of sleeve-shaped neurothekeoma of the ulnar nerve, which was incidentally discovered during a cubital tunnel release surgery. METHODS: A 57-year-old man was admitted at our institution with clinical, ultrasonographic, and electromyographic findings highly suggestive of cubital tunnel syndrome...

A 9-year-old golden retriever dog was diagnosed with a left retrobulbar mass. Fine-needle aspirations and incisional biopsies resulted in discordant diagnoses: myxosarcoma/myxoma or rhadomyosarcoma, respectively. Immunohistochemistry following exenteration allowed definitive diagnosis of malignant peripheral nerve sheath tumor with fibromyxomatous differentiation. Fifteen weeks after surgery, an aggressive recurrence resulted in euthanasia.

We present a rare case of intraoral nerve sheath myxoma. Clinically, the neoplasm mimics many other oral mucosal pathosis, underscoring the importance of histopathology in ensuring accurate diagnosis of oral mucosal lesions. Reports of intraoral nerve sheath myxomas are essential to enhance our understanding of this rare intraoral entity.

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Nerve sheath myxomas (NSM) are uncommon benign tumours of nerve sheath origin. These tumours are most often located in the dermis and subcutaneous tissue of the head and neck region, but less frequently can also involve extracutaneous sites but they are rarely seen in the oral cavity. Many authors consider nerve sheath myxoma and neurothekeoma to be variants of the same tumour, though currently it has been demonstrated that both the lesions are distinct entities with different origins and unique morphological features...

OBJECTIVE: To analyze intramuscular soft-tissue tumors with fatty rind, and to evaluate the difference between fatty rind and split fat sign on magnetic resonance imaging (MRI). MATERIALS AND METHODS: We retrospectively analyzed 50 pathologically confirmed intramuscular masses on MRI. We evaluated the distribution and shape of fatty rind and muscle atrophy. RESULTS: Fatty rind was found more frequently in benign lesions (80% [36 out of 45]) compared with malignant lesions (25% [1 out of 5]; P = 0...

Peripheral nerve sheath tumors (PNSTs) are known to occur in the orbit and comprise 4% of all orbital tumors, but have not been well studied in contemporary literature. Ninety specimens involving the eye and ocular adnexa (1979-2015) from 67 patients were studied. The mean age was 32.5years. Locations included orbit (58.9%), eyelid (60.0%), and other ocular adnexa. Most specimens were neurofibromas (70.0%), followed by schwannomas (11.1%), neuromas (11.1%), granular cell tumors (n=4), nerve sheath myxomas (n=2), and malignant PNST (n=1)...

Neurothekeomas (NTKs) are benign cutaneous neoplasms of fibrohistiocytic origin and most commonly occur in the head, neck, and upper extremities. Traditionally, NTK and nerve sheath myxoma (NSM) were classified as subtypes of a single neoplasm with a common histogenesis, but recently immunostaining has demonstrated that the lesions are most likely of distinct cellular origin. Rarely, NTKs have been reported to occur in the ocular adnexa, and the present case of a 39-year-old female is the first to describe a cellular NTK originating in the cornea and mimicking a Salzmann's nodular degeneration...

Objective: To investigate the clinicopathologic characteristics and differential diagnosis of dermal nerve sheath myxoma (DNSM) and neurothekeoma (NTK). Methods: Clinical, pathological features and immunohistochemical profiles in 9 cases of DNSM and 8 cases of NTK were comparatively studied. The literature was reviewed. Results: The involved site of 9 DNSMs included the hand/fingers ( n =3), ear ( n =2), face, back, abdominal wall and waist (1 case each). Two of 8 NTKs arose in the forearm ( n =2), one each in the nose, lip, shoulder, supraclavicular region, leg and hand...

Oral neurothekeoma or nerve sheath myxoma is a rare benign oral tumour of nerve sheath origin. Historically, this tumour has been subclassified as myxoid (classic), mixed, or the cellular type, depending on the amount of myxoid stroma and cellularity. We present a case of oral neurothekeoma (mixed type) of the buccal mucosa. The tumour was completely excised. No recurrence was detected in the last 3 years after local excision.

Herein we present the case of a 16-month boy, clinically diagnosed with infantile digital fibromatosis, but 9 months after continued growth, the mass was excised and the histopathologic diagnosis was that of a benign nerve sheath myxoma. We present this case to emphasize that nerve sheath myxomas (also known as myxoid neurothekeoma) should be included in the differential diagnosis of dermal nodules in infants.