Pathology of desmoplastic trichoepithelioma dr sampurna roy md. It is a stromal change that in the context of dysplasia supports the diagnosis of an invasive carcinoma. Desmoplastic trichoepithelioma is a harmless facial skin lesion. Trichoepithelioma an overview sciencedirect topics. Basal cell carcinoma mimicking desmoplastic trichoepithelioma. A brazilian study of several types of cutaneous tumors reported only a partial response for trichoepithelioma to 5% imiquimod cream. Trichoepithelioma is regarded as a poorly differentiated hamartoma of the hair germ. The tumour islands of syringoma are often comma shaped and have been. Soeprono teaches and practices dermatopathology at loma linda university, school of medicine, department of dermatology. However, bcc may display overlapping histopathologic findings with. Merkel cells are integral constituents of desmoplastic. In contrast to trichoepithelioma, it does not appear to be a feature of multiple trichoepithelioma syndromes. The tumour is comprised of small islands of basaloid epithelium superficially which form cords and strands towards the base and periphery figures 6, 7. Anubha bajaj, department of histopathology, panjab.
Unlike trichoepithelioma which may be multiple, desmoplastic trichoepithelioma is typically solitary and found in middleaged women, almost always appearing on the face the cause and risk factors for desmoplastic trichoepithelioma are unknown. Narrow strands of basaloid cells, desmoplastic stroma, and keratinous cysts, and is associated with ossification in approximately 6% of cases. Histopathology was characteristic, with the exophytic desmoplastic trichoepithelioma surrounded by the melanocytic proliferation, and supported the hypothesis that trichoepithelioma is an acquired condition, probably induced by the release of. A type of nodular vertical growth melanoma with scanty spindle cells, prominent desmoplastic stroma and often minimal atypia. Other studies suggest the possibility of targeted therapies, such as antitumor necrosis factor.
An unusual variant designated immature trichoepithelioma has also been described. Aug, 2010 desmoplastic trichoepithelioma is an uncommon benign neoplasm of follicular derivation that usually manifests as a sporadic asymptomatic small indurated annular lesion with a raised border and a. Trichoepithelioma arising in a congenital melanocytic nevus. Diagnostic value of cd34 immunostaining in desmoplastic trichilemmoma. Read desmoplastic trichoepithelioma versus morphoeic basal cell carcinoma. Trichoepithelioma arising in a congenital melanocytic. Benign, resembles basal cell carcinomamorphea type. Currently, there are no known methods to prevent desmoplastic trichoepithelioma occurrence. Hundreds of clinical and histopathologic images can be found throughout this practical reference. Syringoma exhibits striking ductal differentiation in the centre of the tumour islands.
Desmoplastic trichoepithelioma is benign tumor and can be managed safely with surgical removal, electrodesiccation and curettage. Differential expression of phlda1 tdag51 in basal cell. Trichoepithelioma is a benign neoplasm that shares both clinical and histological. Desmoplastic trichoepithelioma the term desmoplastic trichoepithelioma was introduced in 1977 by brownstein and shapiro in a series of 50 cases. Its sweat gland or sebaceouslines of differentiation have been also reported. Epithelioma adenoides cysticumtrichoepithelioma ecronicon. Autosomal dominant related tumors are multiple, semitransparent, domeshaped papules on face, scalp, neck, upper trunk. Desmoplastic trichoepithelioma is a rare benign adnexal tumor that usually presents as an asymptomatic, firm, annular plaque. Sep 25, 2018 trichoepithelioma is a benign adnexal neoplasm. These are the most common symptoms of trichoepithelioma.
Histopathology images of desmoplastic small round cell. Junker institute of pathology, klinikum bremenmitte, school of medicine of the university of gttingen, bremen, germany background desmoplastic trichoepithelioma dt is a. Jun 01, 2008 read desmoplastic trichoepithelioma versus morphoeic basal cell carcinoma. The cells in desmoplastic trichoepithelioma are suggested to be in close association with the basal cells in the outer root sheath.
Skin adnexal tumors in plain language archives of pathology. Bernard ackerman, md histopathologic clues help the user arrive at a diagnosis. It has also been called sclerosing epithelial hamartoma. Trichoepithelioma pictures, treatment, symptoms, causes. It is also known as the desmoplastic response, desmoplastic stroma, desmoplastic stromal response, and stromal response. Trichoepithelioma pictures, types, symptoms, treatment. Discussion desmoplastic trichoepithelioma is an uncommon benign adnexal tumour with an incidence estimated to be 2. Laboratory of dermatopathology, port washington, ny.
The tumor cells form hair follicles without any hair shafts. Pathology of desmoplastic trichoepithelioma dr sampurna. Treatment of the trichoepithelioma lesion is primarily surgical. It almost develops as a solitary lesion, although rare. Histopathology was characteristic, with the exophytic desmoplastic trichoepithelioma surrounded by the melanocytic proliferation, and supported the hypothesis that trichoepithelioma is an acquired condition, probably induced by the release of growth factors and cytokines from melanocytes. Histologic mimics of basal cell carcinoma archives of. Syringoma desmoplastic trichoepithelioma can be differentiated by the presence of horn cysts, follicular differentiation, calcification and the presence of long epithelial strands, all of which are uncommonly seen in syringoma. Jul 31, 20 dermatopathology reference describes trichoepithelioma histopathology including histologic features and provides links to additional medical references.
Less commonly it might be localized on neck, upper trunk area and scalp. After establishing the diagnosis, we performed surgical extirpation of the scar with a 2 mm margin for assessment. Desmoplastic trichoepithelioma and intradermal nevus. A case of desmoplastic trichoepithelioma with ossification. Desmoplastic trichoepithelioma although most classifications include this as a separate entity ackerman and his colleagues have suggested that such tumours should be grouped together with. Immunohistochemistry in dermatopathology tammie ferringer, md context. Soepronos textbook is available on and includes detailed information on over 600 entities and includes four dvd diskettes with highresolution images that provide a virtual dermatopathology reference and guide. How can desmoplastic trichoepithelioma be prevented. A dt of the upper lip was diagnosed histologically by a small. Desmoplastic trichoepithelioma or sclerosing epithelial hamartoma. Background a recent small series demonstrated perfect sensitivity and specificity utilizing immunostaining for phlda1, a marker of follicular stem cells, in the distinction of desmoplastic trichoepithelioma and morphoeiform basal cell carcinoma bcc in small biopsy specimens objectives to assess this result more broadly methods we performed immunoperoxidase staining of bccs superficial n. Nineteen cases of dte were compared histologically and. Desmoplastic trichoepithelioma is a benign neoplasm considered to have follicular differentiation. Brookespiegler syndrome, familial cylindromatosis, and.
Clinically, it typically shows 38 mm diameter, annular, yellowish or skincolored asymptomatic plaques, with an elevated border and a depressed or atrophic center on face 1. The stroma surrounding these aggregates characteristically has moderate number of fibroblasts1. Trichoepitheliomas are mostly seen in the scalp, nose, forehead, and upper lip. The aim was to clarify which criteria for differentiation offer the best basis for diagnosis. Trichoepithelioma is a neoplasm of the adnexa of the skin. It primarily occurs in the abdominal cavity associated with mesentery and peritoneum and presents as large abdominal mass with peritoneal effusion. The rapid growth and aggressive histologic features often observed in these tumors can cause diagnostic uncertainty and they are often confused with basal cell carcinoma. Histopathology was characteristic, with the exophytic desmoplastic trichoepithelioma surrounded by the melanocytic proliferation, and supported the hypothesis that trichoepithelioma is an acquired condition, probably induced by the release of growth. Histopathological and immunohistochemical findings from incisional. Desmoplastic trichoepithelioma dte has cords of banal cells and. Desmoplastic trichoepithelioma definition of desmoplastic. We studied desmoplastic trichoepitheliomas associated with intradermal nevi. Trichoepithelioma its sweat gland or sebaceouslines of differentiation have been also reported. Extensive fibrous stroma surrounds epithelial islands.
It is often difficult to differentiate between cases of desmoplastic trichoepithelioma dte and morphoeic basal cell carcinoma mbcc because both lesions have many features in common. A desmoplastic trichoepithelioma is a cutaneous condition characterized by a solitary, firm skin lesion on the face 672. It is an uncommon form of trichoepithelioma, with a reported incidence of 1 in 5000 skin biopsies in a cohort of british adults. Immunohistochemistry is not a diagnostic test but a highly valuable tool that requires interpretation within a context.
What is the prognosis of desmoplastic trichoepithelioma. The biopsy showed no residual tumour and the cosmetic result was good. Dermatopathology reference describes desmoplastic trichoepithelioma histopathology including histologic features and provides links to additional medical references. Jul 21, 2015 desmoplastic trichoepithelioma dte is a rare benign adnexal tumor with the characteristic features of asymptomatic, solitary, annular, indurated and centrally depressed papules or plaques, most commonly occurring in younger individuals on the face. May 23, 2011 desmoplastic trichoepithelioma dte is an uncommon benign adnexal tumor that usually present as flesh colored solitary, annular, indurated and centrally depressed papule or plaque on the face. Desmoplastic trichoepithelioma versus morphoeic basal cell carcinoma. It typically arises as a slowgrowing papule, often with a central dell, on the face with a predi. Histologic mimics of basal cell carcinoma archives of pathology. To enhance learning, the clues are followed by answers, explanations, and differential diagnoses.
The histopathology is similar to classical trichoepithelioma, which is characterized by horn cysts and aggregates of basaloid cells in superficial dermis. The tissue microarrays were performed using a manual tissue arrayer. Desmoplastic trichoepithelioma dte is an uncommon adnexal tumor usually found on the. However, hypocellular desmoplastic stroma, a unique feature of. Sellheyer, p75 neurotrophin receptor differentiates between morphoeic basal cell carcinoma and desmoplastic trichoepithelioma. Desmoplastic trichoepithelioma dte is a histologic variant of te that merits additional description. Desmoplastic trichoepithelioma dte is an uncommon adnexal tumor usually found on the face of young women. Laser and radiofrequency have been used with diverse results.
A desmoplastic trichoepithelioma is a cutaneous condition characterized by a solitary, firm skin lesion on the face. It most commonly occurs on sunexposed areas in middleaged women. Diagnostic utility of immunohistochemistry in distinguishing. Desmoplastic small round cell tumor desmoplastic small round cell tumor. It is a distinct variant of trichoepithelioma based upon its unique clinical and histopathological characteristics 1. Histopathology images of desmoplastic small round cell tumor. Pdf trichoepithelioma is a benign neoplasm with hair follicle differentiation that. Trichoepitheliomas may be divided into the following types.
Cases associated with brookespiegler syndrome are caused by mutations of the cylindromatosis oncogene cyld, which maps to 16q12q. It is characterized by a proliferation of small, solid islands of basaloid cells showing invaginations resembling dermal hair papillae and lacking obvious keratinous cyst formation see fig. Brookespiegler syndrome, familial cylindromatosis, and multiple. The lesion is unique and it also has a firm texture. Desmoplastic trichoepithelioma aka sclerosing epithelial hamartoma presents as a solitary asymptomatic slow growing lesion on face cheek, chin, or forehead and neck of young adults fm. Jan 21, 2014 trichoepithelioma is a rare benign skin lesion that originates from hair follicles. Desmoplasia is the formation of fibrous connective tissue.
Scanning power view demonstrates an infiltrating tumour which appears to be arising from the epidermis figures 4, 5. Desmoplastic trichoepithelioma dte is a histologic variant of te that. Trichoepithelioma is a rare benign skin lesion that originates from hair follicles. We sought to describe the clinical and histologic characteristics of dte and discuss management strategies for these tumors. Special attention was given to tumors with follicular differentiation. Despite the use of panels of immune markers, histopathological criteria. We present a large family of iranian origin with 15 individuals affected with multiple familial trichoepithelioma in four generations, and treated with three different methods. Microcystic adnexal carcinoma of the upper lip misdiagnosed benign desmoplastic trichoepithelioma jan rustemeyer 0 stefan zwerger 0 matthies prksen 0 klaus junker 0 0 k. The clinical criteria, histopathological features and immunohistochemical. It is a firm annular lesion, white or yellow in appearance, with a depressed center and elevated edges. Desmoplastic trichoepithelioma versus morphoeic basal cell. Histologically, desmoplastic trichoepithelioma has three characteristic findings. Desmoplastic trichoepithelioma with perineural involvement.
Desmoplastic trichoepithelioma dte is a rare benign adnexal tumor with the characteristic features of asymptomatic, solitary, annular, indurated and centrally depressed papules or plaques, most commonly occurring in younger individuals on the face. Pathology outlines trichoepithelioma trichoblastoma. The histological diagnosis can be difficult in some cases. In the present study we examined the localization of merkel cells in another adnexal tumor, the desmoplastic trichoepithelioma n 15, as well as in its main differential diagnosis, the morpheiform basal. Desmoplastic trichoepithelioma dt is a benign appendageal tumour predominately localized on the facial skin. Desmoplastic trichoepithelioma is an uncommon adnexal tumor that appears to have a predilection for the face of young women. Desmoplastic trichoepithelioma although most classifications include this as a separate entity ackerman and his colleagues have suggested that such tumours should be grouped together with trichoblastomas and use the term trichoblastoma for all tumours showing predominant hair germ differentiation. Desmoplastic trichoepithelioma grows from cells lining the hair follicle. Typically, dte presents as a firm plaque or nodule with a central dell on the face of young to middleaged individuals. Histologically, dte is characterized by thin cords of small basaloid cells in a densely sclerotic stroma figure 3, d. It is also known as the desmoplastic response, desmoplastic stroma, desmoplastic stromal response, and stromal response it is a stromal change that in the context of dysplasia supports the diagnosis of an invasive carcinoma. Desmoplastic trichoepithelioma dte is an uncommon benign adnexal tumor that usually present as flesh colored solitary, annular, indurated and centrally depressed papule or plaque on the face.
Desmoplastic trichoepithelioma wiley online library. With an incidence of 1 in 5000 skin biopsies in adults, it is reported more. Desmoplastic trichoepithelioma dte is a rare benign adnexal tumor. Desmoplastic trichoepithelioma this type is characterized by the presence of a single lesion on the face. Cells are often in fronds, may have 2 or more layers of basaloid cells, may have papillary mesenchymal bodies. Desmoplastic trichoepithelioma is an uncommon benign neoplasm of follicular derivation that usually manifests as a sporadic asymptomatic small indurated annular lesion with a raised border and a. Partial malignant transformation of a dt is a rarity and a complete transformation has never been described in literature.