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Cutaneous Neurocristic Hamartoma

MedGen UID:
882657
•Concept ID:
C4053530
•
Disease or Syndrome

Definition

A rare dermal and subcutaneous lesion that results from the aberrant development of neural crest-derived melanocytes. It affects the head and neck with a predilection for the scalp. It presents as a slowly enlarging multinodular plaque. Morphologically is characterized by the presence of melanocytes and structures with schwannian differentiation in the dermis and subcutaneous tissue. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCutaneous Neurocristic Hamartoma

Recent clinical studies

Etiology

Large plaque-type blue naevus with subcutaneous cellular nodules.
Skowron F, Balme B
Clin Exp Dermatol 2009 Dec;34(8):e782-4. Epub 2009 Oct 9 doi: 10.1111/j.1365-2230.2009.03511.x. PMID: 19817763

Diagnosis

Cutaneous Neurocristic Hamartoma Mimicking Basal Cell Carcinoma in a Patient With Xeroderma Pigmentosum.
Finberg A, Martin S, Gradecki S, Zlotoff B, Raghavan SS
Am J Dermatopathol 2022 May 1;44(5):e54-e56. doi: 10.1097/DAD.0000000000002133. PMID: 35170472
Extensive neurocristic hamartoma with bone marrow involvement.
Conrad DM, Chaplin A, Walsh NM, Pasternak S
Am J Dermatopathol 2010 Jul;32(5):486-8. doi: 10.1097/DAD.0b013e3181c3422b. PMID: 20414093
Large plaque-type blue naevus with subcutaneous cellular nodules.
Skowron F, Balme B
Clin Exp Dermatol 2009 Dec;34(8):e782-4. Epub 2009 Oct 9 doi: 10.1111/j.1365-2230.2009.03511.x. PMID: 19817763

Prognosis

Cutaneous Neurocristic Hamartoma Mimicking Basal Cell Carcinoma in a Patient With Xeroderma Pigmentosum.
Finberg A, Martin S, Gradecki S, Zlotoff B, Raghavan SS
Am J Dermatopathol 2022 May 1;44(5):e54-e56. doi: 10.1097/DAD.0000000000002133. PMID: 35170472
Large plaque-type blue naevus with subcutaneous cellular nodules.
Skowron F, Balme B
Clin Exp Dermatol 2009 Dec;34(8):e782-4. Epub 2009 Oct 9 doi: 10.1111/j.1365-2230.2009.03511.x. PMID: 19817763

Clinical prediction guides

Cutaneous neurocristic hamartoma presenting as cutis verticis gyrata.
Saha D, Kini UA, Kini H
Am J Dermatopathol 2014 Mar;36(3):e66-9. doi: 10.1097/DAD.0b013e3182919c76. PMID: 23812020

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