Hamartoma mesenquimal rabdomiomatoso

Bernal-Mañas, C M; Isaac-Montero, M.A.; Vargas-Uribe, M.C.; Ruiz-Pruneda, R.; Hernández-Bermejo, J.P.

doi:10.1016/j.anpedi.2012.08.005

Cited by 3 publications

(5 citation statements)

References 10 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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“…The mean size of our STHs was 5.7 mm (±3.6), which is in keeping with most of the reported cases which are ≤1 cm, 14,36,37,40,49,52,55,57,63,64 with only one of our cases being >1 cm. However, multiple cases have been reported measuring >1 cm 38,39,41‐43,45‐47,50,51,53,54,58 with the largest case to our knowledge measuring 4.9 × 3.5 × 3 cm 60 …”

Section: Discussionmentioning

confidence: 90%

“…Histopathologically the lesions were characterized by striated muscle proliferation on the dermis. Since then more than 60 cases have been reported in the literature, with a preference for pediatric patients, especially for newborns and infants, arguing for a true congenital nature to this lesion 13,36‐58 . To date, the largest case series described five cases, with three cases presenting in the pediatric age and the remaining two cases presenting in a 48‐ and a 54‐year‐old patients 14 .…”

Section: Discussionmentioning

confidence: 99%

“…Clinically, SMHs muscle hamartomas have a broad presentation ranging from a skin tag, 13,14,37,38,43,49,50,58 a mass-forming lesion, 36,40,41,44,45,51,53,[55][56][57]60,61 a papule/plaque/nodule, 14,39,[46][47][48]54,59,[62][63][64] a polypoid mass, 52 and a cystic lesion. 42 Of our six cases with clinical description, available two cases presented as papule/plaque, two as a mass, one as a skin tag, and one as a pigmented lesion.…”

Section: Striated Muscle Hamartomamentioning

confidence: 99%

“…47 The mean size of our STHs was 5.7 mm (±3.6), which is in keeping with most of the reported cases which are ≤1 cm, 14,36,37,40,49,52,55,57,63,64 with only one of our cases being >1 cm. However, multiple cases have been reported measuring >1 cm 38,39,[41][42][43][45][46][47]50,51,53,54,58 with the largest case to our knowledge measuring 4.9 × 3.5 × 3 cm. 60 Although the name hamartoma implies a benign anomalous proliferation of normal elements to the local site, one prior case of striated muscle hamartoma was reported to recur after 5 months of excision but information regarding the margin status of the primary resection was not provided.…”

Section: Striated Muscle Hamartomamentioning

confidence: 99%

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Smooth muscle hamartoma and striated muscle hamartoma: Clinicopathologic characterization of two rare entities and literature review

González

Dehner

2020

J Cutan Pathol

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Smooth muscle hamartoma (SMH) and striated muscle hamartoma (STH) are anomalous proliferations of smooth muscle or striated muscle, respectively, in anatomic sites where these tissues are normally present. To date, only limited cases have been reported describing these lesions. In this study, we sought to characterize the clinicopathologic features of both SMH and STH. A total of 27 cases of SMH and 12 cases of STH from 1990 to 2020 were identified. SMH cases had a slight male predominance (63%) and a mean age of presentation of 20 years (range: 4 months‐91 years), with a mean size of 9.3 mm (±13.3). In contrast, STH were equally distributed in gender, with a mean age of presentation of 40 years (range: 3 months‐66 years) and a mean size of 5.7 mm (±3.6). SMH were more commonly located in the torso and extremities (70%) and STH in the head and neck area (92%). One case of SMH recurred after 1.1 years and in the initial diagnosis the lesion was present at the tissue edge. None of the cases of STH had a recurrence. We present the largest cohort of SMH and STH, and report the first case of a recurrent SMH, suggesting the importance of obtaining a clean margin for these lesions.

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Biopsy‐Proven Spontaneous Regression of a Rhabdomyomatous Mesenchymal Hamartoma

Mazza

Linnell

Votava

et al. 2014

Pediatric Dermatology

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Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare, benign, congenital tumor of the dermis and subcutaneous tissue comprised of skeletal muscle and adipose and adnexal elements. Although the majority of cases are an incidental finding in otherwise healthy patients, some have been reported in association with other anomalies. We present a full-term boy evaluated on day 2 of life for two lesions located on the midline of the lower back and right buttock that each appeared clinically as an atrophic, pink plaque. Ultrasound of the midline lesion revealed an underlying lipomyelomeningocele with a tethered cord in the spinal canal. Histopathology of the right buttock cutaneous lesion was consistent with a diagnosis of RMH. Surgical excision was performed on the midline intradural lipoma and the lesion on the buttock was monitored clinically. Repeat biopsy of this site at 1 year of age revealed complete spontaneous regression. This case highlights three interesting features: the association with an occult spinal dysraphism lipomyelomeningocele and tethered cord, the clinical presentation of an atrophic plaque as opposed to the more commonly reported raised lesions, and the phenomenon of spontaneous regression of the lesion. Most importantly, this final feature of regression in our patient suggests that, in the absence of symptoms, clinical observation of RMH lesions is warranted for spontaneous regression for 1 to 2 years provided that no functional deficit is noted and that the cutaneous or deeper lesions are not causing a medical problem.

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Hamartoma mesenquimal rabdomiomatoso

Cited by 3 publications

References 10 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

Smooth muscle hamartoma and striated muscle hamartoma: Clinicopathologic characterization of two rare entities and literature review

Biopsy‐Proven Spontaneous Regression of a Rhabdomyomatous Mesenchymal Hamartoma

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