Unusual Recurrent Lateral Canthus Mass in a 16-Year-Old Male Patient: Neurothekeoma (original) (raw)
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Case Rep Oncol Unusual Recurrent Lateral Canthus Mass in a 16-Year-Old Male Patient: Neurothekeoma
Case Reports in Oncology, 2019
Neurothekeoma (NTK) is a specific benign soft tissue tumor, typically involving the skin of the head and neck area as well as the upper part of the body in young age with female predominance. It has a typical lobular pattern of growth but often displays atypical features such as myxoid stroma or fascicular pattern, which makes the diagnosis more difficult and may necessitate the use of immunohistochemical staining to differentiate NTK from nerve sheath tumor. Ocular NTK in general is very rare with only 11 cases previously reported. We are presenting a case of recurrent mixed cellular/myxoid NTK involving the lateral canthal area of a 16-year-old-boy and we demonstrate the diagnostic challenge in such cases to attract the attention of ophthalmologists and pathologist to the rare occurrence of NTK in the ocular region.
Myxoid Neurothekeoma: Report of a rare tumour at unusual site
IP Innovative Publication Pvt. Ltd., 2017
Introduction: Neurothekeoma is a rare benign soft tissue tumor of uncertain histogenesis which affects adolescents and young adults. It is rare to find these tumors occurring in children. Neurothekeoma usually presents with a small, solitary, and slow-growing nodule or papule on the upper body. Case Report: We describe a case of a 9-year-old girl having large (5x4x1) painful swelling over back of 2 months duration. After total resection of the tumor, histological examination and immunostaining confirmed the diagnosis of myxoid neurothekeoma. The clinicopathological features of this rare tumor and its important differential diagnoses are discussed. Conclusion: Neurothekeoma is a rare tumour in children and its' myxoid variant is even rarer. This benign tumour has to be differentiated from other malignant tumors as it has good prognosis.
The American Journal of Surgical Pathology, 2007
This report describes the clinicopathologic findings in 176 patients who presented with 178 tumors currently referred to as neurothekeomas. Our study group included 64 males and 112 females, ranging from 20 months to 85 years old at the time of their first surgical procedure (median age: 17 y). Twenty-four percent of patients were r10 years of age and only 20% of patients were Z30 years of age at initial diagnosis. The patients typically presented with a solitary, superficial, slow-growing, and relatively asymptomatic mass in the 0.3 to 2.0 cm size range. One patient had multiple tumors. More than 75% of the lesions involved the head (n = 63), upper extremities (n = 44), and shoulder girdle (n = 27) regions. The tumors were evident a few weeks to 4 years (median duration: E7 mo) before surgical resection was sought. Histologically, the lesions involved the dermis and/or subcutis, and they formed multinodular masses with varying amounts of myxoid matrix and peripheral fibrosis. On the basis of the amount of myxoid matrix, the tumors were subclassified as cellular (n = 63), mixed (n = 67), or myxoid (n = 48). All cases had spindled and epithelioid mononuclear neoplastic cells with relatively abundant cytoplasm and indistinct cell borders. The majority of cases also had occasional multinucleated tumor cells. The lesional cells had a strong tendency for whorled growth, and oftentimes, focal fascicular growth was also present. Nuclear atypia was minimal in 62 cases, mild in 73 cases, at least focally moderate in 41 cases, and focally marked in 2 cases. Mitotic activity ranged from 0 to 124 mitotic figures/25 wide-field high power fields (WHPFs) (median mitotic count: 4 mitotic figures/25WHPFs). Twenty-five lesions had >10 mitotic figures/25WHPFs. A total of 16 cases (9%) had atypical mitotic figures. Osteoclastlike giant cells were detected in 39% of cases. Immunoreactivity was typically present for vimentin, NKI/C3, CD10, microphthalmia transcription factor, and PGP9.5, and focal reactivity was sometimes noted for smooth muscle actin and CD68. All tumors tested were negative for S100 protein, glial fibrillary acidic protein, and Melan A. The overwhelming majority of cases had involvement of the tissue margins. A complete follow-up record is available for 71 patients (40.3%) with follow-up intervals ranging from 3 years 2 months to 34 years 9 months (median: 17 y 9 mo). Limited or incomplete follow-up information is also available for an additional 14 patients with follow-up intervals ranging from weeks to approximately 10 years (median: 5 mo). Regrowth of tumor after biopsy or local excision was reported in 13 patients, one of whom had 2 recurrences. However, because of the nature of our consultation practice and a tendency for clinicians to specifically send us cases with a complex clinical course, this is believed an overestimation of the true recurrence rate. Neurothekeomas are morphologically and immunohistochemically distinct from true nerve sheath myxomas. An origin from fibroblastic cells with the ability to differentiate into myofibroblasts and a tendency to recruit histiocytic cells is postulated.
Characterization of an intracranial neurothekeoma: case report
Turkish Neurosurgery, 2010
AIm: 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. results: 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. COnClusIOn: 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.
Pediatric cellular neurothekeoma: Seven cases and systematic review of the literature
Pediatric Dermatology, 2020
Cellular neurothekeomas (CNTs) are benign, dermal nodules. 1 The histogenesis of these uncommon lesions is as yet unclear and is a topic of current debate. In 1969, Harkin and Reed first described nerve sheath myxoma, which Gallager and Helwig later characterized as neurothekeomas. 2,3 Neurothekeomas are slow-growing, often asymptomatic dermal tumors, which have traditionally been divided into three subtypes: cellular, myxoid, and mixed. 4 Historically, neurothekeomas and nerve sheath myxomas were synonymous 5 and neurothekeomas were postulated to be of nerve sheath origin. 3 However, recent studies indicate that neurothekeomas may be of fibrohystiocytic differentiation 6 and suggest that nerve sheath myxomas and neurothekeomas are morphologically and histopathologically distinct. 7-9
SM Dermatology Journal, 2023
Neurothekeomas are uncommon benign superficial cutaneous tumors with various histologic patterns, such as myxoid, cellular, or mixed types, depending primarily on the quantity of myxoid matrix present. They generally affect the head and neck, are more common in women than men, and typically start in the second and early third decades of life. Once believed to originate from the nerve sheath, the source is now thought to be fibroblasts that can differentiate into myofibroblasts and entice histiocytes. Neurothekeomas can present a significant challenge for pathologists to diagnose, and strict histomorphologic features and immunohistochemistry studies are essential to establish the diagnosis. We present a recurrent scalp myxoid Neurothekeoma case in a 7-year-old girl and review the pertinent literature.
Facial neurothekeoma in a 10-year-old child
Plastic Surgery, 2007
Neurothekeoma is a rare and benign tumour that occurs most frequently on the head and neck. It is classified as a nerve sheath tumour and epidemiologically occurs in young adults. It is rare to find these tumours occurring in children. The following report describes a case of neurothekeoma in a young girl, its treatment and a review of the literature. Specific histological analysis may reveal atypical and aggressive features, which, in turn, affect resection and reconstructive options.
Myxoid neurothekeoma: a rare soft tissue tumor of hand in a male toddler
Nigerian journal of surgery : official publication of the Nigerian Surgical Research Society, 2013
Neurothekeomas are rare benign neoplasms, typically occurring in young patients with a remarkable predilection for the female population. Patients usually present with a small nodule in different anatomical sites, commonly involving the face and the upper limb. We report a case of a three-year-old boy, who presented with a nontender nodule on the left thumb. Surgical biopsy and immunostaining confirmed the diagnosis as myxoid neurothekeoma. The rarity of this unusual skin tumor in a toddler prompted the following report.