Cutaneous Neural Neoplasms: A Practical Guide

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These are divided into basaloid, clear or eosinophilic and tumors with glandular differentiation. Trichoepithelioma is a benign tumor that can occur as a sporadic, solitary lesion or an autosomal dominant familial disorder known as Brooke—Spiegler syndrome. Histologically, it is a well-circumscribed and symmetric lesion that predominantly consists of uniform basaloid cells with peripheral palisading, surrounded by dense stroma that contains fibroblasts forming papillary mesenchymal bodies. In contrast with basal cell carcinoma BCC , artefactual stroma and myxoid stroma are not present Figures 6 and 7.

Immunohistochemically, trichoepithelioma expresses scattered Merkel cell cytokeratin 20 positivity, while BCC is positive for androgen receptor AR. Desmoplastic trichoepithelioma DTE is characterized by narrow, compressed strands of basaloid germinative epithelium, embedded in densely sclerotic stroma within the upper dermis. Figure 6 Types of tumors predominantly without connection to epidermis.

Trichoblastoma is a benign neoplasm differentiated toward the trichoblast. Histologically, it is a large, circumscribed basaloid tumor that shows less follicular differentiation than trichoepithelioma Figure 7C. Spiradenoma is a benign dermal neoplasm that can show either eccrine or apocrine differentiation. Pain is one of the main clinical characteristics of spiradenoma. It can rarely be associated with Brooke—Spiegler syndrome.

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Microscopically, the tumor consists of one large, sharply demarcated lobule, but more commonly there are several lobules located in the dermis without connections to the epidermis. At a higher magnification, two distinct populations of neoplastic epithelial cells can be seen: cells with small, dark nuclei located at the periphery of the cellular aggregates and the other type of cells with large, pale nuclei located in the center of the aggregates.

T-lymphocytes are usually identified within the tumor Figures 7E, F. The nests of cylindroma are commonly surrounded by a rim of densely eosinophilic PAS-positive basement membrane material. High power shows same cytological features of spiradenoma. Spiradenocarcinoma is a malignant adnexal neoplasm resulting from malignant transformation of a benign spiradenoma.


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In all cases, there are recognizable areas of a benign spiradenoma. The malignant part shows loss of nodular growth pattern, infiltrative borders, nuclear atypia, and tumor necrosis. Pilomatrixoma pilomatricoma, calcifying epithelioma of Malherbe is a benign lesion with differentiation toward the matrix of the hair follicle.

Histopathologically, the appearance varies according to the age of the lesion. There are two basic cell types, basophilic cells and eosinophilic shadow cells. These shadow mummified cells form from the basophilic cells, and the transition may be relatively abrupt or may take place over several layers of cells transitional cells Figures 7H, I. Pilomatrix carcinoma is the malignant counterpart of pilomatricoma. Histologically, it differs from pilomatricoma in asymmetrical growth pattern, poor circumscription, and significant nuclear atypia.

Focal intracytoplasmic pink trichohyaline granules are seen that serve to differentiate pilomatrix carcinoma from other basophilic poorly differentiated malignant tumors. Dermal duct tumor is variant of poroma that is located largely or entirely within the dermis, where it consist of variously shaped tumor islands containing ductal lumina. Please refer to the to the previous poroma section. Sebaceous tumors including adenoma, sebaceoma and carcinoma can be either epidermal or dermal locations. Please refer to the next sebaceous tumors discussion.

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Hidradenoma is a benign adnexal neoplasm, closely related to poroma, that displays a limited degree of ductal differentiation. Histologically, it is a dermal neoplasm with a nodular, circumscribed pattern at scanning magnification. The intervening stroma is vascularized. It is composed of two types of cells: clear and eosinophilic cells. Clear cells contain abundant glycogen, and their number varies from lesion to lesion.

Eosinophilic cells are polygonal with a central vesicular nucleus. Focal cystic spaces are commonly seen Figure 8. Syringomas are small benign adnexal neoplasms that are almost always multiple. By far, the most common sites of involvement are the lower eyelids.


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Histologically, it is restricted to the upper reticular dermis and is composed of numerous small solid nests, cords, and tubules of epithelial cells within a dense stroma. In some lesions, clear cells predominate, and this pattern has been termed clear cell syringoma; it has frequently been associated with diabetes mellitus. Mixed tumor chondroid syringoma is biphasic tumor with both epithelial and stromal components.

The epithelial part is composed of elongated branching tubular structures and may have follicular differentiation. Chondroid, mucoid, or fibrous stroma is usually seen Figures 9C, D. Hidradenoma papilliferum is a benign neoplasm that almost always develops in the vulval and perianal regions of middle-aged women. Estrogen receptors, progesterone receptors, and ARs are commonly expressed in hidradenoma papilliferum. Tubular adenoma tubular papillary adenoma is a benign neoplasm demonstrating apocrine differentiation that typically occurs in women on the scalp region.

The tumor consists of multiple irregularly shaped tubular structures that have a double- to several-layered epithelial lining.

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Many types of carcinoma with sweat gland differentiation show tubular and glandular architectures. They include microcystic adnexal carcinoma, tubular carcinoma, malignant mixed tumor, mucinous carcinoma, digital papillary carcinoma, adenoid cystic carcinoma ACC , and apocrine carcinoma. Microcystic adnexal carcinoma is a locally infiltrative and destructive low-grade adenocarcinoma differentiated toward ducts. Histologically, it is a poorly circumscribed tumor composed of epithelial cords that invades deeply into the dermis and exhibits prominent perineural and intraneural invasion.

The neoplastic cells are small, uniform and basaloid, or less commonly clear and have no or minimal cytological atypia and mitotic activity. Digital papillary carcinoma was historically divided histologically into aggressive digital papillary adenomas and digital adenocarcinomas. However, cases originally classified histologically as adenoma developed metastases, demonstrating that histologic parameters do not accurately predict behavior or allow distinction between adenoma and adenocarcinoma.

Therefore, the term aggressive digital papillary adenoma has been abandoned in favor of classification of all such lesions as digital papillary carcinoma. It is present almost exclusively on the fingers, toes, palms, and soles. Characteristic histologic findings of this lesion include tubuloalveolar and ductal structures associated with papillary projections protruding into cystically dilated lumina.

Mucinous carcinoma is characterized by large pools of basophilic mucin, which are compartmentalized by delicate fibrous septa, thereby creating a honeycomb pattern.

ACC is a very rare malignant tumor that is indistinguishable from its salivary gland counterpart. Sebaceous glands are outgrowths of the external root sheath of the follicle that secrete an oily substance, sebum, into the follicular space.

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Epithelial membrane antigen EMA immunohistochemistry is positive in mature sebaceous cells. There are many non-sebaceous tumors that show focal sebaceous differentiation such as BCC, trichoblastoma, and poroma Figure Enlarged sebaceous gland size and increased number of lobule: sebaceous hyperplasia. The lesions of sebaceous hyperplasia nearly always occur on the face. They consist of a single greatly enlarged sebaceous gland composed of numerous lobules grouped around a centrally located hair follicle Figures 10 and 11A.

Normal sebaceous gland opens directly to skin surface: nevus sebaceous, Fordyce spots, and normal histology in certain areas. Nevus sebaceus of Jadassohn is nearly always located on the scalp or the face as a single lesion and is present at birth. Histologically, the sebaceous glands in nevus sebaceus follow the pattern of normal sebaceous glands during infancy, childhood, and adolescence.

In the first few months of life, they are well developed. During childhood, the sebaceous glands in nevus sebaceus are underdeveloped and, therefore, greatly reduced in size and number. Thus, the diagnosis of nevus sebaceus may be missed. However, the presence of incompletely differentiated hair structures is typical of nevus sebaceus.

There often are cords of undifferentiated cells resembling the embryonic stage of hair follicles. At puberty, the lesion assumes its diagnostic histologic appearance. Large numbers of mature or nearly mature sebaceous glands open directly into the skin surface Figure 11B. Outcomes of primary cutaneous squamous cell carcinoma with perineural invasion: an year cohort study.

Sentinel lymph node biopsy in cutaneous squamous cell carcinoma: a systematic review of the English literature. Dermatol Surg.

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PubMed Google Scholar. Prognostic factors for local recurrence, metastasis, and survival rates in squamous cell carcinoma of the skin, ear, and lip. Implications for treatment modality selection. Perineural invasion in squamous cell skin carcinoma of the head and neck.