Nodular subcutaneous mass arising underneath a congenital nevus

A 14-year-old girl presented with a congenital nevus on the thigh, which had been present from birth and gradually increased in size. Recently, an indurated area underneath the nevus had developed. Physical examination revealed a homogeneous, brown, sharply demarcated hairy patch on the lateral left thigh (Figure 1a). Deep to this was a non-tender palpable mass without erythema or swelling of the surrounding tissue. Following the initial presentation, an ultrasound was performed; this demonstrated a well circumscribed, nodular hypoechoic, inhomogeneous mass (12.8–15.6 mm) in the deep dermis/hypodermis, with posterior enhancement (Figure 1b). Doppler ultrasonography revealed central and peripheral blood vessels of different calibers (Figure 2a, b). Elastography showed increased rigidity of the mass, with a strain ratio of 3.24, indicative of a potential malignant process (Figure 2c). The patient underwent en bloc excision of the tumor. Macroscopically, a firm, pigmented, well circumscribed and lobulated nodule was seen (Figure 3a, b). Histology revealed fascicles of spindle cells associated with blood vessels, stromal hemorrhage, nodular and diffuse lymphoplasmacytic infiltrate and hemosiderin deposits (Figure 3c). Immunohistochemistry showed diffuse CD68, desmin-positive and CD31-negative tumoral cells, S100 positivity in isolated dendritic cells as well as EMA-positive plasmocytes. Nodular subcutaneous mass arising underneath a congenital nevus Case for Diagnosis

[1]  G. Metzler,et al.  Patterns of infiltration and local recurrences of various types of cutaneous sarcomas following three‐dimensional histology , 2018, Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG.

[2]  T. Jacques,et al.  Melanoma in congenital melanocytic naevi , 2017, The British journal of dermatology.

[3]  Kenichi Saito,et al.  Angiomatoid fibrous histiocytoma: a series of seven cases including genetically confirmed aggressive cases and a literature review , 2017, BMC Musculoskeletal Disorders.

[4]  C. Guarneri,et al.  Unexpected amelanotic melanoma simulators , 2017, Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG.

[5]  S. Bolboacă,et al.  Doppler ultrasound and strain elastography in the assessment of cutaneous melanoma: preliminary results. , 2015, Medical ultrasonography.

[6]  R. Sciot,et al.  Angiomatoid fibrous histiocytoma – case series with emphasis on a late fibrotic variant , 2015, Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG.

[7]  C. Fletcher,et al.  WHO classification of tumours of soft tissue and bone , 2013 .

[8]  A. Mansfield,et al.  Angiomatoid fibrous histiocytoma in a 25-year-old male , 2010, Rare tumors.

[9]  E. Montgomery,et al.  Utility of FISH in the diagnosis of angiomatoid fibrous histiocytoma: a series of 18 cases , 2010, Modern Pathology.

[10]  C. Fletcher The evolving classification of soft tissue tumours: an update based on the new WHO classification , 2006, Histopathology.

[11]  D. Arber,et al.  Angiomatoid Fibrous Histiocytoma , 1996, Annals of plastic surgery.

[12]  M. Kransdorf,et al.  Benign soft-tissue tumors in a large referral population: distribution of specific diagnoses by age, sex, and location. , 1995, AJR. American journal of roentgenology.

[13]  M. Kransdorf Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location. , 1995, AJR. American journal of roentgenology.

[14]  Michael J. Costa,et al.  Angiomatoid Malignant Fibrous Histiocytoma: A Follow‐up Study of 108 Cases with Evaluation of Possible Histologic Predictors of Outcome , 1990, The American journal of surgical pathology.

[15]  F. Enzinger Angiomatoid malignant fibrous histiocytoma. A distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm , 1979, Cancer.