Possible Causes for Psammomatous Melanotic Schwannomas Carney Complex Type 1 melanotic schwannoma (PMS). [kjim.org] Malignant psammomatous melanotic schwannoma of the spine: A component of Carney complex. [surgicalneurologyint.com] Histopathology: The soft tissue schwannomas are a very unusual variant known as psammomatous melanotic schwannoma. [dermatologyadvisor.com] Carney Complex We present a case of psammomatous melanotic schwannoma as part of Carney complex in a 67-year-old male. [ncbi.nlm.nih.gov] Histopathologic examination revealed the diagnosis of a malignant psammomatous melanotic schwannoma that is associated with the Carney complex in 50% of these patients. [ncbi.nlm.nih.gov] Psammomatous melanotic schwannoma as a component of Carney complex. [ijpmonline.org] Carney-Stratakis Syndrome Although rare, cancerous tumors may occur in Carney complex including thyroid carcinoma and malignant psammomatous melanotic schwannoma. [rarediseases.org] Ghosh, Psammomatous melanotic schwannoma as a component of Carney complex. Indian J. Pathol. [springermedizin.de] Carney: Psammomatous melanotic schwannoma. A distinctive inheritable tumor with special associations, including cardiac myxoma and endocrine overactivity. [whonamedit.com] Carney Triad melanotic schwannoma testicular tumors Sertoli cell tumors : most common Pathology Carney complex has autosomal dominant inheritance related to mutation of the PRKAR1A tumor [radiopaedia.org] melanotic schwannoma Blue nevus, epithelioid blue nevus (multiple) Breast ductal adenomata (multiple) Osteochondromyxoma Supplemental criteria Affected first-degree relative [bigtomato.org] […] also well recognized including: extracardiac myxoma breast testis thyroid brain adrenal gland: primary pigmented nodular adrenocortical disease (PPNAD) pituitary adenoma psammomatous [radiopaedia.org] Primary Pigmented Nodular Adrenocortical Disease melanotic schwannomas (5%); also neurofibromatosis, cerebral hemangioma]; associated with 2p16 abnormalities Sporadic and non-familial patients are usually infants or age [pathologyoutlines.com] The psammomatous melanotic schwannomas are typically benign; they metastasize, however, in as many as 10% of cases. [emedicine.medscape.com] Carney Psammomatous melanotic schwannoma. [elsevier.es] Blue Nevus melanotic schwannoma. [histopathology-india.net] Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. [emedicine.medscape.com] […] of skin and mucous membrane (Example: Epithelioid type blue nevi or usual type of blue naevi), cardiac myxoma, endocrine overactivity syndromes and neurinomas including psammomatous [histopathology-india.net] Sertoli Cell Tumor of the Testis melanotic schwannoma, blue nevus, ductal adenoma of breast, and osteochondromyxoma. [jpatholtm.org] melanotic schwannoma. [path.upmc.edu] […] mucosal myxoma, myxomatosis of the breast, primary pigmented nodular adrenocortical disease, acromegaly with growth hormone-producing adenoma, LCCSCT, thyroid carcinoma, psammomatous [jpatholtm.org] Bednar Tumor It has to be distinguished from other pigmented cutaneous spindle cell lesions like pigmented neurofibroma, psammomatous melanotic schwannoma, neurocristic cutaneous hamartoma [ncbi.nlm.nih.gov] Primary Pigmented Nodular Adrenocortical Disease 4 […] pituitary gland tumor schwannoma (neurilemmoma), peripheral nerve schwannoma of the bone schwannoma, psammomatous melanotic schwannomas of the (posterior) spinal nerve roots [familialcancerdatabase.nl] melanotic schwannomas (5%); also neurofibromatosis, cerebral hemangioma]; associated with 2p16 abnormalities Sporadic and non-familial patients are usually infants or age [pathologyoutlines.com] The psammomatous melanotic schwannomas are typically benign; they metastasize, however, in as many as 10% of cases. [emedicine.medscape.com] Primary Pigmented Nodular Adrenocortical Disease 1 […] pituitary gland tumor schwannoma (neurilemmoma), peripheral nerve schwannoma of the bone schwannoma, psammomatous melanotic schwannomas of the (posterior) spinal nerve roots [familialcancerdatabase.nl] melanotic schwannomas (5%); also neurofibromatosis, cerebral hemangioma]; associated with 2p16 abnormalities Sporadic and non-familial patients are usually infants or age [pathologyoutlines.com] The psammomatous melanotic schwannomas are typically benign; they metastasize, however, in as many as 10% of cases. [emedicine.medscape.com] Pleural Neurilemmoma benign myxoid schwannoma, benign epithelioid schwannoma, benign glandular schwannoma, and psammomatous melanotic schwannoma.[2] Case Report A 60-year-old woman presented [digitmedicine.com] […] such as leiomyoma, leiomyosarcoma, and calcifying aponeurotic fibroma which may also show palisading of the nuclei.[2] Histopathological variants of this lesion are ancient schwannoma [digitmedicine.com] Mediastinal Neurofibroma Psammomatous melanotic schwannoma localized in the mediastinum: diagnosis by fine-needle aspiration cytology. Diagn Cytopathol 1998;19:298-302. [med.amegroups.com] Familial Atrial Myxoma […] pituitary gland tumor schwannoma (neurilemmoma), peripheral nerve schwannoma of the bone schwannoma, psammomatous melanotic schwannomas of the (posterior) spinal nerve roots [familialcancerdatabase.nl] Although rare, cancerous tumors may occur in Carney complex including thyroid carcinoma and malignant psammomatous melanotic schwannoma. [rarediseases.org] melanotic schwannomas, vi) primary pigmented nodular adrenocortical disease (including patients symptomatic with Cushing syndrome), vii) testicular tumours (bilateral, multicentric [histopathology-india.net] Spindle Cell Sarcoma of the Duodenum Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. [nature.com] In general, most psammomatous melanotic schwannomas behave indolently, but rare extraintestinal examples have had a more aggressive clinical course. 27 It is unclear if the [nature.com] Figure 5 Psammomatous melanotic schwannoma. ( a ) The tumor is seen here occupying the mucosa and submucosa and is composed of bland spindle to polygonal plump, bland cells [nature.com] Ureter Schwannoma Cutaneous psammomatous melanotic schwannoma: non-recurrence with surgical excision. Am J Clin Dermatol. 2003;4:799–802. [5]. Arun G, Chakraborti S, Rai S, et al. [journals.lww.com] Multiple schwannoma of the seminal vesicle: A case report. Medicine. 2020;99:e21603. [4]. Claessens N, Heymans O, Arrese JE, et al. [journals.lww.com] Neurilemmoma In psammomatous melanotic schwannoma, varying numbers of psammoma bodies are present. [histopathology-india.net] [8] and a variant with a plexiform multinodular pattern. [9] Other variants are associated with genetic syndromes such as Carney complex (ie, psammomatous melanotic schwannoma [emedicine.medscape.com] melanotic schwannoma); neurofibromatosis (NF) type 2 (NF2, ie, cranial or spinal root neurilemmoma; multiple cutaneous plexiform schwannoma [11] ; neurilemmomatosis (schwannomatosis [emedicine.medscape.com] Sertoli Cell Tumor melanotic schwannoma. [path.upmc.edu] […] myxoma, myxoid mammary fibroadenoma, spotty pigmentation, primary pigmented nodular adrenocortical disease, testicular tumor, growth hormone secreting pituitary tumor, and psammomatous [path.upmc.edu] Clear Cell Sarcoma melanotic schwannoma, and metastatic MM.[7] The distinction between CCS and MM should be done [Table 1]. [ncbi.nlm.nih.gov] melanotic schwannoma, and metastatic MM.[ 7 ] The distinction between CCS and MM should be done [ Table 1 ]. [ncbi.nlm.nih.gov] […] melanocytic tumor, clear cell myomelanocytic tumor, malignant peripheral nerve sheath tumor, monophasic synovial sarcoma, deep-seated epithelioid sarcoma, adult fibrosarcoma, psammomatous [ncbi.nlm.nih.gov] Meningeal Melanocytoma REFERENCES: Carney JA (1990) Psammomatous melanotic schwannoma. [path.upmc.edu] Carney JA (1990) Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. [docslide.com.br] Fine-needle aspiration diagnosis of psammomatous melanotic schwannoma. Diagn Cytopathol. 2000;23:55-8 [ Pubmed ]. [npplweb.com] Congenital Non-Neoplastic Nevus Epithelioid blue nevus and psammomatous melanotic schwannoma: the unusual pigmented skin tumors of the Carney complex. Semin Diagn Pathol 1998 ; 15 ( 3 ): 216–24. 48. [cambridge.org] PDE4D Haploinsufficiency Syndrome schwannomas. [uniprot.org] Disease description CNC is a multiple neoplasia syndrome characterized by spotty skin pigmentation, cardiac and other myxomas, endocrine tumors, and psammomatous melanotic [uniprot.org] Zosteriform Lentiginosis Carney, J.A.: Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. [bioscience.org] A multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol 20, 259-272 (1996) 28. [bioscience.org] In addition, three new components of the syndrome have been identified: psammomatous melanotic schwannoma, epithelioid blue nevus, and ductal adenoma of the breast (26, 37 [bioscience.org] Juvenile Nevus Epithelioid blue nevus and psammomatous melanotic schwannoma: the unusual pigmented skin tumors of the Carney complex. Semin Diagn Pathol 1998 ; 15 ( 3 ): 216–24. 48. [cambridge.org] Hypotrichosis - Lymphedema - Telangiectasia with Membranoproliferative Glomerulonephritis melanotic schwannoma Carvajal Syndrome Cutaneous findings • Striate epidermolytic palmoplantar keratoderma; wooly scalp hair Genetics • AR, desmoplakin mutations Associations [plasticsurgerykey.com] […] adrenal gland ; p/w primary pigmented nodular adrenocortical disease → Cushing’s • Other endocrine abnormalities: pituitary adenomas and testicular cancer (Sertoli type) • A/w psammomatous [plasticsurgerykey.com] Cardiac Metastasis melanotic schwannoma, breast ductal adenoma, and osteochondromyxoma. [merckmanuals.com] […] adrenocortical disease causing Cushing syndrome, growth hormone and prolactin-producing pituitary adenoma, testicular tumors, thyroid adenoma or carcinoma, and ovarian cysts), psammomatous [merckmanuals.com] Malignant Perineurioma Psammomatous melanotic schwannoma 86. [slideshare.net] This variant is more common in schwannomas of the central nervous system. 177 Another type of schwannoma is the psammomatous melanotic schwannoma, which contains melanin and [plasticsurgerykey.com] Melanotic psammomatous M9541/0 Melanotic neurofibroma M9550/0 Plexiform neurofibroma Plexiform neuroma M9560/0 Neurilemoma, NOS Schwannoma, NOS Neurinoma Acoustic neuroma [en.wikipedia.org] Gastrocutaneous Syndrome Carney, J.A.: Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. [bioscience.org] A multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol 20, 259-272 (1996) 28. [bioscience.org] In addition, three new components of the syndrome have been identified: psammomatous melanotic schwannoma, epithelioid blue nevus, and ductal adenoma of the breast (26, 37 [bioscience.org] Cardiac Myxoma melanotic schwannomas, vi) primary pigmented nodular adrenocortical disease (including patients symptomatic with Cushing syndrome), vii) testicular tumours (bilateral, multicentric [histopathology-india.net] […] single or multiple) iii) mammary myxoid fibroadenomas (single or multiple) iv) spotty mucocutaneous pigmentation (including lentigines and blue nevi and combinations) v) psammomatous [histopathology-india.net] Lamellar Ichthyosis melanotic schwannomas psammomatous melanotic schwannomas multiple fractures thin skin blue sclera MVP** Osteogenesis Imperfecta Type 1 Osteogenesis Imperfecta Type II Osteogenesis [brainscape.com] melanotic schwannomas name the findings in LAMB/NAME/Carney complex Lentigenes Atrial Myxoma Mucocutaneous myxomas Blue nevi Nevi Atrial myxomas Myxoid tumors Ephelids psammomatous [brainscape.com] Ferguson Smith, *also found in Muire Torre Syndrome cutaneous myxomas areola myxomas atrial myxomas Cushings syndrome from primary pigmented nodular adrenocortical disease psammomatous [brainscape.com] Benign Lipomatous Neoplasm Psammomatous melanotic schwannoma: a distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. [nejm.org] […] pituitary gland tumour schwannoma (neurilemmoma), peripheral nerve schwannoma of the bone schwannoma, psammomatous melanotic schwannomas of the (posterior) spinal nerve roots [hccpjournal.biomedcentral.com] Epithelioid blue nevus and psammomatous melanotic schwannoma: The unusual pigmented skin tumors of the Carney complex. Semin Diagn Pathol 1998; 15: 216-224. 2. [hccpjournal.biomedcentral.com]
