Background: Noncutaneous psammomatous melanotic schwannoma has recently been reported as an unusual component of Carney's complex (myxomas, spotty pigmentation, and endocrinopathy). The most common locations for this lesion include peripheral nerve roots and the gastrointestinal tract.
Observations: A cutaneous psammomatous melanotic schwannoma is reported in a patient with known Carney's complex. This neoplasm was pseudoencapsulated, with epithelioid to spindle-shaped cells and no nuclear atypia. Immunostaining was positive for S100 protein and vimentin, as well for HMB-45 antibody. Electron microscopy showed melanosomes in cytoplasmic processes of cells that were ensheathed by layers of reduplicated basal lamina.
Conclusions: Location in the superficial soft tissues is extremely unusual for psammomatous melanotic schwannomas. Recognition of this new cutaneous marker as a part of this complex may aid in identification of individuals at risk for cardiac myxomas. It is also important that this lesion is not mistaken for melanoma, given the strong HMB-45 positivity.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1001/archderm.129.2.202 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Spontaneous globe rupture from an intraocular melanotic schwannoma.
Eur J Ophthalmol
November 2024
Department of Ophthalmology, SUNY Upstate Medical University, Syracuse, New York, USA.
Introduction: Melanotic schwannoma (MS) is a rare neoplasm composed of Schwann cells with melanosomes in various maturation stages. While MS is typically observed in spinal nerve roots or peripheral nerves, their involvement in intraocular structures is uncommon. Here, we present a case of spontaneous globe rupture as the presenting feature of intraocular extension of a MS.
View Article and Find Full Text PDFNovel mutation in Carney complex: a case report and literature review.
Front Endocrinol (Lausanne)
July 2024
Department of Medical Oncology, Zhangzhou Municipal Hospital, Zhangzhou Municipal Hospital Affiliated of Fujian Medical University, Zhangzhou, China.
Article Synopsis
- Carney complex is a rare genetic syndrome linked to mutations, and a new mutation was identified in a 23-year-old Chinese patient who has a family history of cardiac myxomas.
- The patient presented with symptoms like central obesity, high blood pressure, hyperpigmentation, and high cortisol levels, leading to a diagnosis of Cushing's syndrome.
- Surgical intervention was performed to remove adrenal and retroperitoneal tumors, resulting in improved health outcomes, including weight loss and reduced cortisol levels.
Malignant melanotic nerve sheath tumor with PRKAR1A, KMT2C, and GNAQ mutations.
Free Neuropathol
January 2022
Departments of Pathology and Laboratory Medicine, University of Louisville, Louisville, KY, USA.
Malignant melanotic nerve sheath tumor (MMNST) is a rare and potentially aggressive lesion defined in the 2021 WHO Classification of Tumors of the Central Nervous System. MMNST demonstrate overlapping histologic and clinical features of schwannoma and melanoma. MMNST often harbor mutations, especially within the Carney Complex.
View Article and Find Full Text PDFSporadic spinal psammomatous malignant melanotic nerve sheath tumor: A case report and literature review.
Front Oncol
February 2023
Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico C. Besta, Milan, Italy.
Background: Sporadic Spinal Psammomatous Malignant Melanotic Nerve Sheath Tumor (SSP-MMNST) is a rare subgroup of peripheral nerve sheath tumors arising along the spine. Only a few reports of SSP-MMNST have been described. In this paper, we review the literature on SSP-MMNST focusing on clinical, and diagnostic features, as well as investigating possible pathogenetic mechanisms to better implement therapeutic strategies.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!