Benign fibrous hamartoma of infancy: a case of MR imaging paralleling histologic findings

Stensby, J. Derek; Conces, Miriam; Nacey, Nicholas C.

doi:10.1007/s00256-014-1940-4

Cited by 23 publications

(11 citation statements)

References 24 publications

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“…Radiologically, FHI is described as having a heterogeneous appearance on MRI, with strands of T1 hyperintense fat that are interspersed with enhancing, T2 hyperintense tissue, consisting of fibrous and mesenchymal or myxoid soft tissues, consistent with findings in our case. While our patient has had no infiltrative disease into the bone, her imaging shows significant bony remodeling and muscular atrophy in the area of the FHI.…”

Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

“…The differential diagnosis for indurated subcutaneous plaques in the newborn period encompasses benign and malignant entities, including but not limited to cystic hygromas, hemangiomas, neurofibromas, fibrolipomas, juvenile fibromatosis, myofibromas, congenital desmoid tumors, fibrosarcomas, and rhabdomyosarcomas. [6][7][8] The histologic diagnosis is very important in differentiating among pathologic entities. FHI diagnosis criteria outlines the presence of three components in various proportions including (a) tive, and the adipocytes are S100 protein positive.…”

Section: Discussion and Con Clus I Onmentioning

confidence: 99%

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Fibrous hamartoma of infancy associated with bony remodeling and muscle atrophy

Kozlowski

Murati

Ogilvie

et al. 2019

Pediatric Dermatology

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Fibrous hamartoma of infancy (FHI) is a benign, poorly demarcated, unencapsulated hamartoma contiguous with surrounding fat. This case highlights pronounced bony remodeling and muscle atrophy associated with FHI in a 6‐week‐old girl. Emphasis is placed on pathologic diagnosis and mechanisms, treatment considerations, and the multidisciplinary approach utilized in the management of the disorder.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Section: Discussion and Con Clus I Onmentioning

confidence: 99%

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Fibrous hamartoma of infancy associated with bony remodeling and muscle atrophy

Kozlowski

Murati

Ogilvie

et al. 2019

Pediatric Dermatology

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“…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. 41 Although isolated cases of fibrous hamartoma of infancy have been reported in patients with tuberous sclerosis 22 and Williams syndrome, 45 these appear to represent chance occurrences, rather than true syndromic association. The overwhelming majority of previously reported cases of fibrous hamartoma of infancy have been cured by simple excision, although~15% have recurred locally in a non-destructive fashion, 55 and rare cases have been reported to recur more than once.…”

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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“…In addition, the study was complemented with FISH for ETV6 present in infantile fibrosarcoma, which was also negative. The use of nuclear magnetic resonance as a complementary diagnostic element has recently been reported, showing an organized fat-like structure with heterogeneous soft tissue interleave bands 16 .…”

Section: Discussionmentioning

confidence: 99%

Características clínicas y radiológicas de una cohorte de niños con trombosis venosa intracraneal

Muftoz

Bustos

Manfredi

2018

Rev. chil. pediatr.

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Introduction: Fibrous hamartoma of infancy (FHI) is a benign, soft tissue tumor that usually occurs in children and has a characteristic histological morphology. Objective: To describe a case of congenital FHI with atypical histological and clinical characteristics. Clinical case: Full-term male newborn, with no perinatal morbid history was referred to dermatology due to a congenital erythematous plaque in the umbilical region. The histological study showed a fusocelullar proliferation in dermis and hypodermis of biphasic distribution, with an infiltrative, swirling pattern and bundles of spindle fibroblast-like and myofibroblast-like cells, associated in depth with a mature adipose tissue component. The immunohistochemical study revealed diffuse positivity for CD34, and focal positivity for FXIIIa, without immunoreactivity for actin, desmin, MyoD1, S100, HMB45, Melan-A, or EMA. Fluorescent in situ hybridization (FISH) was negative for platelet-derived growth factor receptor beta (PDGFR-beta) and for ETV6 gene. PDGFR-beta and ETV6 gene are present in congenital dermatofibrosarcoma protuberans and infantile fibrosarcoma, respectively. This history, in addition to previous histological findings, supported the diagnosis of FHI. Surgical resection was performed, without signs of recurrence during clinical follow-up. Conclusion: It is important to consider the FHI within the differential diagnosis of subcutaneous tumors in children, especially in those under two years of age. Although its behavior is benign, it is similar to multiple benign and malignant lesions, which makes it imperative to perform a histological study in front of suspicious clinical lesions.

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Benign fibrous hamartoma of infancy: a case of MR imaging paralleling histologic findings

Cited by 23 publications

References 24 publications

Fibrous hamartoma of infancy associated with bony remodeling and muscle atrophy

Fibrous hamartoma of infancy associated with bony remodeling and muscle atrophy

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

Características clínicas y radiológicas de una cohorte de niños con trombosis venosa intracraneal

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