Rhabdomyomatous Mesenchymal Hamartoma of Nasal Vestibule

Kang, Ju Wan; Park, Heae Surng; Kim, Jeong Hong

doi:10.1097/scs.0b013e3182903357

Cited by 8 publications

(12 citation statements)

References 7 publications

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“…Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare malformation with 62 reported cases through 2014 . It was first described in 1986 by Hendrick et al as a striated muscle hamartoma and was later termed RMH by Mills et al in 1989 .…”

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confidence: 99%

Rhabdomyomatous mesenchymal hamartoma presenting as a sacral skin tag in two neonates with spinal dysraphism

et al. 2015

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Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital malformation involving the dermis and subcutaneous tissue, of which there were 62 reported cases through 2014. We report RMH in two neonates presenting as a sacral skin tag. In both cases, magnetic resonance imaging (MRI) of the spine showed evidence of spinal dysraphism, including a lipomyelomeningocele and a tethered cord. Surgical repair of the defects was performed. Histopathologic examination of the skin tags showed a haphazard arrangement of mature skeletal muscle fibers and adnexal elements, consistent with RMH. The second patient also had a hemangioma on the sacrum and was diagnosed with LUMBAR (lower body hemangioma and other cutaneous defects, urogenital anomalies/ulceration, myelopathy, bony deformities, anorectal/arterial anomalies, and renal anomalies) syndrome, an association between cutaneous infantile hemangiomas of the lower body and regional congenital anomalies. The apparent association of paraspinal RMH with spinal dysraphism suggests that aberrant migration of mesodermally derived tissues (including skeletal muscle fibers) during neural tube development may be responsible for the pathologic findings in the skin. Additional study of patients with spinal dysraphism and congenital cutaneous lesions may further support this hypothesis.

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confidence: 99%

Rhabdomyomatous mesenchymal hamartoma presenting as a sacral skin tag in two neonates with spinal dysraphism

et al. 2015

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“…RMH usually presents as a solitary skin lesion of the head and neck, particularly on the nose, chin, periorbital, and anterior neck regions. It has also been reported to occur in unusual sites, including the vagina , toe , oral cavity, nasal vestibule , and anal margin . Although typically a raised lesion, one case of a depressed skin lesion has been reported .…”

Section: Discussionmentioning

confidence: 99%

Congenital Papule on the Anterior Neck

Marvin

Guerrero

Tang

et al. 2016

Pediatric Dermatology

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“…Although RMH is a lesion, which typically occurs in the newborns, some cases have been reported as congenital lesions [10,11] or diagnosed in adult patients [12][13][14]. Apart from the skin of the head and neck region, RMH has also been reported in unusual sites, including oral cavity [15][16][17], nasal vestibule [18] and vagina [19]. Clinically, it can present as small dome-shaped papule or a polypoid pedunculated lesions [1][2][3][4][5][6][15][16][17][18][19].…”

Section: Introductionmentioning

confidence: 99%

“…Apart from the skin of the head and neck region, RMH has also been reported in unusual sites, including oral cavity [15][16][17], nasal vestibule [18] and vagina [19]. Clinically, it can present as small dome-shaped papule or a polypoid pedunculated lesions [1][2][3][4][5][6][15][16][17][18][19]. Notably, rare cases of RHM may undergo spontaneous regression [20,21].…”

Section: Introductionmentioning

confidence: 99%

Rhabdomyomatous mesenchymal hamartoma (RMH) of the anal region: an unusual location for such a rare lesion

Longo¹,

Musumeci²,

Amore³

et al. 2014

J Histol Histopathol

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Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare tumour-like lesion, which occurs in the skin of newborns, principally of the face and neck. RMH has also been reported in unusual sites, including oral cavity, nasal vestibule and vagina. In this case report we describe a rare case of RMH arising as a nodular mass at the anal margin of a male newborn. This is an unusual site for such a rare lesion. The histological examination of the lesion showed the typical features of RMH. Desmin and S100 expression has been determined by immunohistochemical analyses to assess the distribution of the mature skeletal muscle cells and small-sized nerves, which represented the principal components of the nodular mass. Our case emphasizes the possibility that RMH may occur in the perianal region and awareness of this possibility is crucial for pathologists to avoid confusion with other skeletal muscle-containing lesions, especially rhabdomyosarcoma.

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Rhabdomyomatous Mesenchymal Hamartoma of Nasal Vestibule

Cited by 8 publications

References 7 publications

Rhabdomyomatous mesenchymal hamartoma presenting as a sacral skin tag in two neonates with spinal dysraphism

Rhabdomyomatous mesenchymal hamartoma presenting as a sacral skin tag in two neonates with spinal dysraphism

Congenital Papule on the Anterior Neck

Rhabdomyomatous mesenchymal hamartoma (RMH) of the anal region: an unusual location for such a rare lesion

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