Epidermal nevus and rhabdomyosarcoma

Dimond, R. L.

doi:10.1001/archderm.112.10.1424

Cited by 10 publications

(10 citation statements)

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“…Nevus sebaceous and epidermal nevus grouped under organoid nevus has a predisposition to malignancies [14]. Two of the thirty reported sialoblastoma cases, seemingly developed from an existing cutaneous hamartoma [4,15].…”

Section: Discussionmentioning

confidence: 99%

Treatment of metastatic sialoblastoma with chemotherapy and surgery

Scott

Krishnan

Bourne

et al. 2007

Pediatric Blood & Cancer

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Tumors of the salivary gland are very uncommon in children. Sialoblastoma is a rare, aggressive, blastomatous, and potentially malignant congenital tumor. Distant metastases are rare. We present a case of sialoblastoma with lung metastases that developed in a 4‐year‐old girl adjacent to a congenital nevus in the left cheek. The tumor was inoperable at diagnosis but the largest of the pulmonary metastases was removed surgically. The patient responded well to chemotherapy and underwent surgical excision of the primary tumor, followed by three more courses of chemotherapy. Pediatr Blood Cancer 2008;50:134–137. © 2006 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 99%

Treatment of metastatic sialoblastoma with chemotherapy and surgery

Scott

Krishnan

Bourne

et al. 2007

Pediatric Blood & Cancer

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“…One of our patients with keratinocytic nevus and systemic involvement developed a paratesticular rhabdomyosarcoma ipsilateral to the nevus. Dimond and Amon (29) reported a patient with epidermal nevus on the neck and trunk following Blaschko lines, in addition to a rhabdomyosarcoma of the bladder. This patient also had café au lait macules with pigmentary nevi on their surfaces.…”

Section: Discussionmentioning

confidence: 99%

Epidermal Nevus Syndromes: Clinical Findings in 35 Patients

Cruz¹,

Tamayo‐Sánchez²,

Durán‐McKinster³

et al. 2004

Pediatric Dermatology

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Of the patients with epidermal nevi, 10-18% may have disorders of the eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms.

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“…Various extracutaneous malignancies have been reported in patients with EN, including astrocytoma, glioma, Wilms’ tumor, nephroblastoma, salivary gland carcinoma, bladder carcinoma, rhabdomyosarcoma, and gastrointestinal carcinomas . Although many EN have mutations in genes associated with tumor formation and suppression, the specific mutations found in EN are often different from those found in tumors.…”

Section: Further Evaluation and Managementmentioning

confidence: 99%

Epidermal nevus syndromes: New insights into whorls and swirls

Asch

Sugarman

2017

Pediatric Dermatology

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Knowledge of the molecular underpinnings of many epidermal nevi and epidermal nevus syndrome has expanded rapidly in recent years. In this review and update on epidermal nevus syndrome, we will cover recent genetic discoveries involving epidermal nevi, including nevus sebaceus, keratinocytic epidermal nevus, nevus comedonicus, congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome, phakomatosis pigmentokeratotica, Becker's nevus, porokeratotic adnexal ostial nevus, inflammatory linear verrucous epidermal nevi, and cutaneous-skeletal hypophosphatemia syndrome. We will discuss how newly defined mutations relate to the biology reflected in the cutaneous patterns seen in these mosaic disorders and how new molecular data has informed our understanding of these diseases and shaped management decisions. We will discuss the more well-delineated EN and ENS, including keratinocytic EN, nevus sebaceus, nevus comedonicus (NC), congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD) syndrome, phakomatosis pigmentokeratotica, Becker's nevus, porokeratotic adnexal ostial nevus (PAON), inflammatory linear verrucous EN (ILVEN), and the newly described cutaneous-skeletal hypophosphatemia syndrome (CSHS). We will examine advances in the genetics of these disorders and highlight promising work in the translation to management for our patients. We will also briefly discuss less well-defined EN and ENS associated with overgrowth syndromes.

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Epidermal nevus and rhabdomyosarcoma

Cited by 10 publications

References 0 publications

Treatment of metastatic sialoblastoma with chemotherapy and surgery

Treatment of metastatic sialoblastoma with chemotherapy and surgery

Epidermal Nevus Syndromes: Clinical Findings in 35 Patients

Epidermal nevus syndromes: New insights into whorls and swirls

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