Cytologic diagnosis of fibrous hamartoma of infancy - A case report of a rare soft tissue lesion

Department of Pathology, Maulana Azad Medical College and Hindu Rao Hospital, New Delhi, India.
Acta cytologica (Impact Factor: 1.56). 03/2008; 52(2):201-3. DOI: 10.1159/000325480
Source: PubMed

ABSTRACT Fibrous hamartoma of infancy (FHI) is a rare, benign lesion seen in the first 2 years of life. Despite adequate histopathologic descriptions, reports of it in the cytology literature are rare.
A 6-month-old male infant presented with an asymptomatic nodule in left anterior axillary fold. The nodule was noticed 6 weeks before presentation. On local examination, it was mobile and rubbery. Considering the endemicity, a presumptive clinical diagnosis of tuberculous lymphadenitis was made. Fine needle aspiration yielded moderately cellular smears composed of short spindle cells in a greasy background admixed with a few adipocytes. No mitosis or abnormal chromatin pattern was seen. In view of the young age of the patient, a cytologic impression of benign lesion or hamartoma was made. Excision of the nodule and histopathologic examination revealed it as FHI.
The cytologist should be aware of the cytologic features of FHI, so that in the proper clinical setting this diagnosis can be made with certainty. The mixture of adipose tissue and bland spindle cells is helpful to differentiate this lesion from other infantile soft tissue lesions.

  • [Show abstract] [Hide abstract]
    ABSTRACT: The era of Open Access (OA) publication, a platform which serves to better disseminate scientific knowledge, is upon us, as more OA journals are in existence than ever before. The idea that peer-reviewed OA publication leads to higher rates of citation has been put forth and shown to be true in several publications. This is a significant benefit to authors and is in addition to another relatively less obvious but highly critical component of the OA charter, i.e. retention of the copyright by the authors in the public domain. In this study, we analyzed the citation rates of OA and traditional non-OA publications specifically for authors in the field of cytopathology.
    CytoJournal 01/2014; 11:10. DOI:10.4103/1742-6413.131739
  • [Show abstract] [Hide abstract]
    ABSTRACT: Fibrous hamartoma of infancy is a benign soft tissue tumor with a characteristic triphasic organoid histologic appearance. It typically occurs within the first 2 years of life. The usual anatomic locations include the upper extremities, axilla, and upper back. Diagnostic challenges occur when this tumor arises in older children, outside of the usual anatomic sites, or when unusual histologic features are encountered. This study reports 60 cases of fibrous hamartoma of infancy from institutional and consultation files. All had a triphasic organoid histologic pattern, but half also displayed an unusual pseudoangiomatous histologic pattern. The male to female ratio was 2.0 (40 boys, 20 girls), with a mean age of 1.5 years (range, 16 d to 8 y) at diagnosis. Tumor size ranged from 0.5 to 9 cm, with a mean of 3.7 cm. Sites included the trunk (40 cases), extremities (17 cases), and head and neck (3 cases). All cases had triphasic elements of mature fibrous tissue, mature adipose tissue, and immature mesenchymal tissue in varying proportions, with the additional pseudoangiomatous pattern in 32 cases. Immunohistochemical analysis demonstrated reactivity for smooth muscle actin and CD34 in the mature fibrous tissue, S100 protein in the mature adipose tissue, and variable CD34 reactivity in immature mesenchymal and pseudoangiomatous foci. Ki-67 proliferative activity was noted in the immature mesenchymal and pseudoangiomatous foci, and Bcl-2 reactivity was restricted to mesenchymal and pseudoangiomatous foci. Follow-up information in 12 cases revealed no evidence of recurrence in 10 patients and local recurrence in 2 patients, each at 3.5 years after primary excision. This study demonstrates an expanded age range (up to 8 y) and anatomic distribution (30 cases outside of the classic locations of the upper extremities, axilla, and upper back) of fibrous hamartoma of infancy. The pseudoangiomatous morphologic variation can lead to challenges in diagnosis and may reflect a maturational phenomenon from the immature mesenchymal component.
    The American journal of surgical pathology 03/2014; 38(3):394-401. DOI:10.1097/PAS.0000000000000104
  • [Show abstract] [Hide abstract]
    ABSTRACT: To discuss the clinical and pathological features, differential diagnosis and prognosis of fibrous hamartoma of infancy (FHI), seventeen FHI specimens were analyzed with H&E staining and strepavidin peroxidase (SP) immunohistochemistry to detect distinguishing tissue markers. The long-term outcomes of select cases were also obtained. Among the 17 patients (13 males, 4 females, average age 16 months), FHI manifested as a subcutaneous painless mass, primarily on the back of the neck, the upper arms and buttocks. One recurrence was noted among six follow-up cases. The tumors consisted of three main components: fibrous connective tissue; mature fat; and undifferentiated mesenchymal tissue. Immunohistochemistry revealed that fibrous connective tissue was positive for SMA and actin, mature fat tissue was positive for S-100 protein, and undifferentiated mesenchymal tissue was positive for CD34 and was partially positive for actin and SMA. The tumors were negative for desmin, NSE, bcl-2, β-catenin and Ki-67. In brief, FHI is a benign, fibroblastic/myofibroblastic proliferative lesion. Defined histologic features of FHI as presented here would distinguish FHI from similar invasive tumors including infant fibromatosis, calcifying aponeurotic fibroma, fibrous fatty tumor and embryonal rhabdomyosarcoma. Once clearly identified, FHI is curable with complete resection.
    International journal of clinical and experimental pathology 01/2015; 8(3):3374-7.