Case Of Fibrous Hamartoma Of Infancy Research Articles

Fibrous hamartoma of infancy. Radiologic-pathologic study of 21 cases: 8 with predominant pseudoangiomatous pattern

Fibrous hamartoma of infancy (FHI) is a very rare benign soft tissue lesion that principally affects the axilla, trunk, and upper extremities of children younger than 2 years. It is usually cured by local excision. Histologically, these lesions have a triphasic morphology in an organoid pattern: mature adipose tissue, fibroblastic/myofibroblastic trabeculae, and small round cell nests in a myxoid matrix. However, morphologic variants have recently been described. Focal areas with a pseudoangiomatous pattern have been found in some FHI, but few cases with predominant pseudoangiomatous areas have been previously described in the medical literature. We report 21 new cases of FHI, 8 of them with a predominant pseudoangiomatous pattern. Our cases with a predominant pseudoangiomatous pattern did not present specific radiological findings.

Fibrous hamartoma of infancy (FHI) of the parotid region in a 3-year-old female presenting as a huge parapharyngeal tumor: A very rare case presentation

Introduction: Fibrous hamartoma of infancy (FHI) is a rare, benign tumor arising from the lower dermis and subcutaneous tissue. It usually occurs in children below two years of age and is often misdiagnosed as being of malignant nature. It most commonly effects the lower extremities, very rarely involves the head and neck region, and is commonly found in males. Microscopically, it is a poorly circumscribed and non-capsulated tumor comprising of interlacing fascicles of pale eosinophilic myofibroblasts and stellate shaped fibroblasts with island of mature adipocytes. Case Report: We report a very rare case of FHI of parotid gland in a 3-year-old female child, posing a diagnostic difficulty for management. The site, size, and age of presentation of the tumor were unique. This tumor is common in males but our case was a female child. Our patient presented with involvement of the parotid, submandibular and upper neck area with a huge size of around 7 × 8 cm which was causing mild swallowing difficulty due to intraoral swelling in the child. Cytopathology from the lesion demonstrated spindle cell lesion, the differential diagnosis of which is often malignant in pediatric age group. We took a biopsy for confirmatory diagnosis and after histopathological diagnosis of FHI proceeded with complete surgical excision. Conclusion: Treatment of choice for these benign tumors is complete surgical excision, with excellent prognosis.

Clinicopathological analysis of fibrous hamartoma of infancy: report of 12 cases

Objective To investigate the clinicopathological features and prognosis of fibrous hamartoma of infancy (FHI). Methods Totally, 12 confirmed cases of FHI were collected from 2009 to 2014 at the Department of Dermatology, Beijing Children's Hospital. The clinicopathological features of FHI were analyzed. Results Of the 12 patients, 7 were male and 5 were female. The median age of patients was 8 months (range, 5-20 months) , with a median age at onset of 3.5 months (range, 1-12 months) , a median clinical course of 5 months (range, 1-12 months). Skin lesions were skin-colored or pink subcutaneous nodules or plaques, and were located on the trunk in 5 patients, on the extremities in 6 patients, and on the scrotum in 1 patient. Ten patients had solitary lesions, and two had multiple lesions. The largest diameter of skin lesions varied from 2 to 25 cm. Lesions rapidly progressed in 10 patients, which were soft or hard on palpation, and were usually mistaken for hemangioma, lipoma and fibroma. All the patients were treated by surgical excision. After the operation, relapse occurred in 3 patients, but not in the other 9 patients. After secondary resection, the 3 patients experienced no relapse again. Histopathological examination revealed fibrous tissue and mature fat tissue arranged in an interwoven pattern, as well as mesenchymal cells arranged in concentric circles. The tumor mainly consisted of fibrous tissue and mature fat tissue at varying ratios. Immunohistochemically, the fibrous tissue expressed smooth muscle actin (SMA) , fat tissue expressed S100, and mesenchymal cells expressed CD99. The mesenchymal cells were differentiated into fibrous tissue and fat tissue to different extents. Conclusions FHI often manifests as solitary, rapidly growing benign tumor of soft tissue located on the trunk or extremities, and mostly occurs during the first 6 months of life. The skin manifestations of FHI are usually various, but the pathological changes are characteristic. Surgical resection is usually effective for its treatment. Key words: Hamartoma; Fibrous hamartoma of infancy

Cutaneous changes in fibrous hamartoma of infancy

Fibrous hamartoma of infancy (FHI) is a fast growing soft tissue tumor that usually arises in the first 2 years of life. The histology of the lesion has been well described. Few studies, however, have looked at changes in the overlying skin and its appendages. A database search performed at British Columbia Children's Hospital yielded 15 cases of unequivocal FHI occurring in 12 patients (three were recurrences). Of these, we were able to retrieve 13. Five of 13 cases had sections including epidermis. These slides were reviewed with specific emphasis on skin adnexae. Of the cases with excised epidermis in continuity with the lesion, 5/5 had eccrine changes, including hyperplasia, duct dilatation, intraluminal papillary formations, and squamous syringometaplasia. One case showed epidermal basaloid follicular hyperplasia. This study shows that eccrine changes are frequently seen in cases of FHI when overlying skin is sampled. This may be a useful clue to consider this diagnosis, especially when the biopsy is superficial.

Fibrous Hamartoma of infancy: A report of two cases in the genital region

Objectives. Fibrous hamartoma of infancy (FHI) is an uncommon subcutaneous proliferative lesion usually found in the upper portions of the body, particularly in males during the first 2 years of life. We report 2 cases of FHI in the genital region. Methods. We retrospectively reviewed the charts of children treated at Children's Hospital and Health Center, with FHI located in the genital region. Results. We report 2 cases of FHI in the genital region, 1 involving the scrotum and 1 the labium majus. Conclusions. FHI should be considered in the differential diagnosis of genital masses in children. The histologic features are characteristic, local excision of the tumor is curative, and the local recurrence rate is low.

Fibrous hamartoma of the wrist in infancy

Fibrous hamartoma of infancy (FHI) is a rare, self-limiting, benign tumor that occurs in the early years of life. The vast majority of reported cases occur in the proximal upper extremity and torso. A case of FHI that occurred in the wrist of a 9-month-old girl is presented. Diagnostic features of FHI are discussed and emphasized. Surgical treatment is highly successful and is the method of choice.