Fibrous Hamartomas of the Scalp in Infancy

Bl, Eppley; Harruff, Richard C.; Shah, Muhammad Ghiasuddin; Am, Sadove

doi:10.1097/00006534-199407000-00024

Cited by 6 publications

(8 citation statements)

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“…55 However, cases occurring in older children are well-documented. 4,8 The tumors are much more common in males, and most often present as a painless subcutaneous mass involving the axilla, trunk, upper arm, and external genitalia, although isolated cases of fibrous hamartoma of infancy have been reported in a wide variety of anatomical locations, including the scalp, 16 foot, 35 hand, 24 and buttock. 10 MRI has been reported to show an organized arrangement of fat with interspersed, heterogeneous soft tissue bands that may Fibrous hamartoma of infancy suggest this diagnosis in the appropriate clinical setting.…”

Section: Discussionmentioning

confidence: 99%

Fibrous hamartoma of infancy: a clinicopathologic study of 145 cases, including 2 with sarcomatous features

et al. 2017

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Fibrous hamartoma of infancy is a rare soft tissue lesion of infants and young children with characteristic triphasic morphology, which typically occurs in the axilla and less commonly in other locations. We reviewed 145 cases of fibrous hamartoma of infancy from our consultation archives. Cases occurred in 106 males and 39 females (mean age-15 months; range-birth to 14 years), and involved both typical sites (eg, axilla/back/upper arm) (n=69) and unusual locations (n=76). Six were congenital. The tumors presented as subcutaneous masses and ranged from 0.4 to 17 cm (mean 3 cm). All displayed triphasic morphology, but varied widely in the relative percentages of fat, fibroblastic fascicles, and primitive mesenchyme. Hyalinized zones with cracking artifact, mimicking giant cell fibroblastoma, were present in a 44 (30%) of cases; however FISH for PDGFB gene rearrangement was negative in five tested cases. In addition to classical fibrous hamartoma of infancy, two lesions contained large sarcomatous-appearing foci with high cellularity, high nuclear grade, and brisk mitotic activity. One occurred in a 10-month-old female as a new mass in a congenital fibrous hamartoma of infancy; the other occurred as a leg mass in a 6-year-old male. ETV6 gene rearrangement was negative in the tumor from the 10-month-old female. Genomic microarray (OncoScan) showed normal molecular karyotype in eight tested cases, whereas the two tumors with sarcomatous features showed a hyperdiploid/near tetraploid molecular karyotype with copy neutral loss of heterozygosity of chromosomes 1p and 11p, and loss of 10p, chromosome 14, and a large portion of chromosome 22q (22q11.23q13.33), respectively. Follow-up (52 patients; range: 1-208 months, median: 8 months) showed only two local recurrences and no metastases. Extensive local disease in the 10-month-old female with sarcomatous-appearing fibrous hamartoma of infancy necessitated forequarter amputation. In summary, our study confirms the classic clinicopathologic features, including the triphasic morphologic appearance of most cases. In contrast to earlier studies, our series illustrates a broader histologic spectrum than previously appreciated, including its close resemblance to giant cell fibroblastoma in one quarter of cases and the rare presence of 'sarcomatous' areas, the latter providing evidence that these are complex neoplasms rather than hamartomas.

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Section: Discussionmentioning

confidence: 99%

Fibrous hamartoma of infancy: a clinicopathologic study of 145 cases, including 2 with sarcomatous features

et al. 2017

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“…Cytogenetic abnormalities have been described in rare cases, including a reciprocal translocation t(2;3)(q31; q21) [196] and a complex translocation involving chromosomes 6, 8, and 12 [198].…”

Section: Fibromatosesmentioning

confidence: 99%

“…Almost all are diagnosed by 2 years of age, and there is a male predominance. The solitary subcutaneous mass commonly involves the axillary and inguinal regions, upper arms, upper trunk, and external genitalia, although the head and extremities may be affected [189][190][191][192][193][194][195][196]. There are no known familial or syndromic associations.…”

Section: Fibrous Hamartoma Of Infancymentioning

confidence: 99%

Fibroblastic and Myofibroblastic Tumors in Children and Adolescents

2012

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Fibroblastic and myofibroblastic tumors in children and adolescents are a relatively common group of soft tissue proliferations that range from reactive to hamartomatous to neoplastic, with a full spectrum of benign, intermediate, and malignant neoplasms. These lesions are diagnostically challenging because of morphologic and immunohistochemical overlap, despite significant clinical, genetic, and prognostic differences. The fibromatoses are a major subgroup, and all types of fibromatoses can occur in the 1st 2 decades of life. Intermediate and malignant fibroblastic-myofibroblastic tumors are an important group that includes variants of fibrosarcoma and other tumors with recurrent cytogenetic or molecular genetic abnormalities and low metastatic potential. Pathologic examination is enhanced by adjunct techniques, such as immunohistochemistry, cytogenetics, and molecular genetics, although morphology provides the ultimate criteria for a specific diagnosis. This article reviews the clinicopathologic features of fibroblastic and myofibroblastic tumors with an emphasis on the unique aspects of these neoplasms in children and adolescents, the use of diagnostic adjuncts, and differential diagnoses.

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“…A few cases, however, have also been reported on the distal extremities, head and neck. 1,2,8,9 There is no report with a familial or syndromic association, or a case that occurred in combination with other hamartomas.…”

Section: Discussionmentioning

confidence: 99%

“…It most commonly occurs on the upper body, particularly the axillary region, upper arm, upper trunk, the inguinal region and the external genital area. A few cases, however, have also been reported on the distal extremities, head and neck 1,2,8,9 . There is no report with a familial or syndromic association, or a case that occurred in combination with other hamartomas.…”

Section: Discussionmentioning

confidence: 99%

Fibrous hamartoma of infancy manifesting as multiple nodules with hypertrichosis

Yoon

Kim

2006

The Journal of Dermatology

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Fibrous hamartoma of infancy is a rare benign tumor, which usually occurs within the first year of life. Most cases present as a solitary, painless, skin-colored subcutaneous nodule; multiple lesions are rarely reported. There have also been a few cases which have showed overlying skin changes, including hypertrichosis and alterations in pigmentation. We report a rare case of fibrous hamartoma of infancy in a 6-month-old female who presented as multiple nodules with overlying hypertrichosis on the left buttock. To the best of our knowledge, there have been only two reported cases in the dermatological published work that showed these unusual findings simultaneously.

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Fibrous Hamartomas of the Scalp in Infancy

Cited by 6 publications

References 0 publications

Fibrous hamartoma of infancy: a clinicopathologic study of 145 cases, including 2 with sarcomatous features

Fibrous hamartoma of infancy: a clinicopathologic study of 145 cases, including 2 with sarcomatous features

Fibroblastic and Myofibroblastic Tumors in Children and Adolescents

Fibrous hamartoma of infancy manifesting as multiple nodules with hypertrichosis

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