Characteristic Clinical and Dermoscopic Features of Nonvolar Poroma.

BACKGROUND: A poroma typically presents as a solitary, pink-to-red papule or nodule in acral volar areas. However, in nonvolar areas, this typical clinical feature (TCF) can be difficult to identify. OBJECTIVE: We aimed to compare clinical and dermoscopic characteristics between nonvolar poroma (NVP) and volar (ie, typical) poroma (VP). METHODS: We assessed the clinical and dermoscopic characteristics of 40 patients with poromas who were divided into the NVP and VP groups. RESULTS: Of the 40 patients, 20 (50.0%) were allocated to the NVP group and 20 (50.0%) to the VP group. Pigmented variants were more common in the NVP group than in the VP group (60.0% vs 5.0%). The TCF of poroma was observed less frequently in the NVP than the VP group (45.0% vs 85.0%). Approximately one-third (30.0%) of patients with NVP received an initial clinical diagnosis of skin cancer. Dermoscopic patterns associated with melanoma or basal cell carcinoma were more common in the NVP group than in the VP group (65% vs 30%). CONCLUSIONS: Skin cancer-associated clinicodermoscopic features were more frequently observed in patients with NVP, who simultaneously lost dermoscopic patterns associated to poromas and acquired those associated with skin cancer, than those with VP.

Immunohistochemical Study of Non-Epithelial Cells in Spiradenoma.

BACKGROUND: Intratumoral lymphocytes are a defining feature of spiradenoma; however, there have only been a few reports on the phenotypic features of non-epithelial cells. Spiradenomas also contain numerous cells positive for S-100 protein and the nature of these cells is still controversial. METHODS: We performed a clinicopathological and immunohistochemical study of ten cases of spiradenoma. RESULTS: The study included seven men and three women. On histopathological examination, spiradenoma could be divided into two types: the vascular proliferating (VP) type (five cases) that featured granulation tissue with edema, vascular proliferation, and inflammatory cell infiltration into the stroma, and the common type (five cases), which did not include any of the aforementioned features. Immunohistochemical staining demonstrated a large number of cells positive for S-100 protein. These included cells with large pale nuclei, dendritic cells, and a few cells with small dark nuclei that were also positive for α-smooth muscle actin. Most of the cells infiltrating the parenchymata of these lesions were CD3-positive. The proportions of CD4-positive and CD8-positive cells were almost equal or CD8-positive cells were predominant. CD20+ cells were observed in five spiradenomas. In painful lesions, there were numerous nerve fibers near the tumor. CONCLUSIONS: In spiradenoma, CD3+ T cells were mainly seen in the parenchyma and CD8+ cells were predominant over CD4+ cells in most cases. CD20+ cells showed focal infiltration of the parenchyma and stroma, especially in VP-type lesions. S-100 protein-positive cells in spiradenoma contained not only Langerhans cells, but also cells with myoepithelial differentiation.

Apocrine lesions of the breast: part 1 of a two-part review: benign, atypical and in situ apocrine proliferations of the breast.

Apocrine morphology is a common phenomenon encountered in everyday breast pathology practice, and is defined as cuboidal or columnar cells exhibiting abundant eosinophilic granular cytoplasm, prominent apical granules, a low nuclear-cytoplasmic ratio, and round nuclei with pale chromatin and prominent nucleoli. Apocrine morphology is recognised in benign, atypical and malignant lesions of the breast. The morphology of apocrine atypia and non-high-grade apocrine ductal carcinoma in situ (DCIS) is less well defined due to the relative rarity of these lesions. In part 1 of this two-part review, we focus on the morphological characteristics of benign, atypical and in situ apocrine lesions of the breast, summarise the available data to date regarding distinction of atypical apocrine proliferations from non-high-grade apocrine DCIS and the biological significance of apocrine atypia, and provide practical guidance on handling these difficult lesions. Part 2 of this review will focus on the concept of pure apocrine carcinoma with emphasis on its definition and molecular data, including the current understanding of the molecular apocrine signature in breast carcinoma. We complete the review with a synopsis on the utility of immunohistochemistry in the diagnosis of apocrine lesions of the breast.

Head and neck sweat gland adenocarcinoma: A population-based perspective of a rare entity.

OBJECTIVES: Head and neck sweat gland adenocarcinoma (HNSGA) is an extremely rare malignancy. We present the first population-based analysis regarding this entity. STUDY DESIGN: Retrospective population-based analysis. METHODS: Using the Surveillance, Epidemiology, and End Results registry from 2000 to 2013, we extracted 627 cases of HNSGA. Data were analyzed for incidence trends, demographic and clinicopathologic traits, and predictors of disease-specific survival (DSS). RESULTS: The majority HNSGA cases were white, male, and 60 to 79 years old. The incidence was 0.036 per 100,000 people. Tumors most often presented as localized disease and histological grade II/III. The skin of the face was the most common primary site (43.4%), followed by the scalp and neck (31.6%). Overall 5-, 10-, and 20-year DSS were 94.6%, 89.6%, and 79.8%, respectively. Ethnicity did not affect survival, whereas a younger age at diagnosis and female sex conferred an advantage at 10 years (P = 0.0386) and 5 years (P = 0.0191), respectively. The origin of the HNSGA (apocrine vs. eccrine) did not affect outcomes. Regional and distant disease predicted worse DSS at 5, 10, and 20 years (P = 0.0026, P < 0.001, P < 0.001, respectively). Compared to grade I/II disease, grade III/IV dramatically worsened 5-, 10-, and 20-year DSS (P = 0.0035, P < 0.0001, P = 0.0011, respectively). Scalp and neck HNSGA exhibited the poorest 20-year DSS compared to other primary sites (P = 0.0024). CONCLUSION: We present the largest cohort of HNSGA. Significant poor prognostic indicators include older age, higher tumor grade, greater extent of invasion, and primary site of the scalp or neck. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2757-2762, 2017.

Tubulopapillary Cystic Adenoma With Apocrine Differentiation: A Unifying Concept for Syringocystadenoma Papilliferum, Apocrine Gland Cyst, and Tubular Papillary Adenoma.

Syringocystadenoma papilliferum (SCAP), apocrine gland cyst (AGC, also called apocrine hidrocystoma or apocrine cystadenoma), and tubular papillary adenoma (TPA) with apocrine differentiation are defined as proliferations of apocrine epithelium with myoepithelial cells. At Sapporo Dermatopathology Institute, we retrieved 308 benign neoplastic lesions diagnosed as SCAP, AGC, or TPA and combinations of these entities. Among the 308 lesions, 202 (66%) exhibited features of only one type, of which 144 (47%) were AGC, 39 (13%) were TPA, and 19 (6%) were SCAP. The other 106 lesions (34%) had features of 2 or more types, including 56 lesions that were AGC + TPA (18%), 2 that were AGC + SCAP (1%), 34 that were TPA + SCAP (11%), and 14 that were AGC + TPA + SCAP (5%). The most frequent site of these lesions was the face (56%), followed by the scalp (13%). Lesions with the features of AGC were more frequently found on the face, especially the periocular region, than at other sites. TPA lesions were more frequent on the face and scalp than at other sites, whereas SCAP lesions were preferentially found on the face, scalp, and trunk. We also retrieved clinicopathological data and other information. We propose a unifying concept for AGC, TPA, and SCAP. Approximately one-third of these lesions are composite entities with the features of 2 or 3 different tumors, and we propose calling such tumors tubulopapillary cystic adenoma with apocrine differentiation.

Characteristics of Eccrine Tumors in a Tertiary Institution: A 5-Year Retrospective Study.

Eccrine tumors are adnexal tumors with a varied clinical presentation and wide histological spectrum. This study aims to consolidate data on the clinical characteristics of eccrine tumors to help improve clinical acumen and management of such tumors. Histopathological records from January 2008 to December 2012 were retrieved. Clinical characteristics of the tumor including site, appearance, symptoms, color, duration prior to presentation, and clinical and histological diagnosis were recorded. Eighty-four patients with eccrine tumors were identified, with seven main types of tumors recognized-hidradenoma (33.3%), poroma (29.8%), mixed tumors (14.3%), spiradenoma (8.3%), porocarcinoma (6.7%), eccrine adenoma (3.6%), and syringoma (3.6%). A total of 50% of mixed tumors were misdiagnosed as epidermal cysts. Eccrine tumors have a wide array of clinical presentations and are often clinically misdiagnosed as cysts. Recognizing certain clinical features may aid in the diagnosis, but, if in doubt, a biopsy should be performed.

[Endocrine mucin-producing sweat gland carcinoma. three case reports with a brief review of the literature]. / Endokrines muzinproduzierendes Schweißdrüsenkarzinom. Drei Fallberichte mit kurzer Literaturübersicht.

Endocrine mucin-producing sweat gland carcinoma (EMPS) is a rare low-grade sweat gland carcinoma with an infiltrating growth pattern. It occurs mostly in women and shows a predilection for the periorbital region. Histopathologically, the tumor shows analogous features to endocrine ductal carcinoma/solid papillary carcinoma of the breast and shares some clinical and morphological similarities with primary mucinous carcinoma of the skin. The tumor is characterized by large monomorphous epithelial cells with little nuclear pleomorphism and only a few mitotic figures. The solid cystic tumor shows mucin-filled small cystic spaces, cribriform areas and expresses the neuroendocrine markers synaptophysin, chromogranin and neuron-specific enolase with varying staining intensities. The tumor cells are also positive for estrogen and progesterone receptors. We present three cases of this rare tumor with typical clinical, histopathological and immunohistochemical findings, give a short summary of the literature and discuss the most relevant differential diagnoses.

[Apocrine poroma. A relatively little known skin tumor with multilineage differentiation]. / Das apokrine Porom. Ein wenig bekannter Hauttumor mit adnexieller Mischdifferenzierung.

Poromas were originally classified as eccrine tumors which predominantly consist of poroid ductal cells and differentiate in the direction of sweat gland ducts. However, there have now been many reports on poromas with additional differential characteristics differentiating in the direction of sebaceous and/or apocrine glands and/or hair follicles. These tumors have been termed apocrine poromas. Multilineage differentiation within a poroma can be explained by the embryological association of the sweat duct with the so-called folliculo-sebaceous-apocrine unit. The clinical and histopathological features of apocrine poromas are reviewed in comparison to classical eccrine poromas by taking into account seven own cases of apocrine poroma and a review of the literature. It is important for histopathologists not to confuse apocrine poroma with other tumors with multilineage differentiation. Apocrine poroma needs to be distinguished from sebaceoma and from basal cell carcinoma with sebaceous differentiation, in particular, because these tumors have therapeutic consequences for the patient. The main histopathological differences between apocrine poroma, sebaceoma and basal cell carcinoma with sebaceous differentiation are explained.

Familial eruptive syringomas: case report and review of the literature.

BACKGROUND: Syringomas are benign neoplasms of eccrine origin. A clinical variant is eruptive syringomas, which presents as firm, smooth, yellow to pigmented papules that appear as successive crops on the neck, axillae, chest, abdomen, and/or periumbilical region. To our knowledge, there are only 10 published reports of familial eruptive syringomas. Herein we describe the eleventh report of familial eruptive syringomas, review the literature on this unusual presentation, and suggest a novel classification of familial syringomas based on our literature review. OBSERVATIONS: We report two cases of eruptive syringoma within a family. Eruptive syringomas were widely distributed on the trunk of a healthy 16-year-old female and her 19-year-old brother. Both the 19-year-old man and his mother also had infraorbital syringomas. CONCLUSION: Familial eruptive syringomas are a rare clinical entity that is likely autosomal dominantly inherited. Future reports of this unusual condition may provide further insight into the etiology of familial syringomas, and genetic analysis of cases may enable the causative gene mutation to be determined.

A study of histopathologic spectrum of nodular hidradenoma.

BACKGROUND: Hidradenomas are rare benign adnexal neoplasms that encompass a morphological gamut with a range of differentiation. As a consequence, there is a great likelihood of being mistaken for other primary and metastatic tumors. Though conventionally regarded as eccrine, they have been reclassified into eccrine and apocrine types. OBJECTIVE: This study aims to document the histological spectrum of nodular hidradenomas, with particular reference to categorizing them into eccrine or apocrine tumors. RESULTS: A total of 15 cases with features of nodular hidradenoma with their age ranging from 18 years to 73 years were studied. Most of the cases were solitary, circumscribed, solid and cystic, dermal, symmetrical, lobulated tumors with a sheet-like and papillary architecture. The cells were chiefly eosinophilic with a regular oval grooved nucleus and a small inconspicuous nucleolus. Clear cells were also seen. Squamous differentiation was an important feature, with most showing a infundibular type of keratinization. Sebaceous differentiation is also common. The stroma varied from hyaline to myxoid. Only 1 case showed poroid differentiation. CONCLUSIONS: This study describes the assortment of histologic characteristics in hidradenomas. Apocrine hidradenomas are more common, contrary to earlier belief that favored an eccrine origin.

Immunohistochemical differentiation of four benign eccrine tumors.

BACKGROUND: The histogenesis and differentiation of eccrine tumors, including cylindroma, poroma, spiradenoma and syringoma, remains controversial. This controversy may be because of sporadic and incomplete studies of these neoplasms. METHODS: Ten examples each of normal eccrine structures and of four benign eccrine tumors are analyzed with antibodies to cytokeratin (CK) 7, CD34, CK6, CK10, smooth muscle actin (SMA) and CD10. These markers represent two different immunohistochemical stains for each part of the eccrine structure; CK7 and CD34 stain the secretory coil, CK6 and CK10 stain the straight duct and SMA and CD10 stain the myoepithelial cells. This redundancy in staining is performed on four benign eccrine tumors to better interpret the existing literature. RESULTS: We find that CK7 is a sensitive marker for the secretory coil; both cylindromas and spiradenomas express CK7. We also find that CK6 is a marker for the inner ductal cells, while CK10 is a marker for the middle ductal cells; syringomas express both these markers. SMA appears to be a more specific marker for myoepithelial cells surrounding normal eccrine coils, and none of the studied tumors express SMA or CD10. CONCLUSIONS: Our studies suggest that syringomas are tumors of the eccrine duct, while cylindromas and spiradenomas are tumors of the secretory coil.

Apocrine hidradenocarcinoma of the scalp: a classification conundrum.

INTRODUCTION: The classification of malignant sweat gland lesions is complex. Traditionally, cutaneous sweat gland tumors have been classified by either eccrine or apocrine features. METHODS: A case report of a 33-year-old Hispanic man with a left scalp mass diagnosed as a malignancy of adnexal origin preoperatively is discussed. After presentation at our multidisciplinary tumor board, excision with ipsilateral neck dissection was undertaken. RESULTS: Final pathology revealed an apocrine hidradenocarcinoma. The classification and behavior of this entity are discussed in this report. CONCLUSION: Apocrine hidradenocarcinoma can be viewed as an aggressive malignant lesion of cutaneous sweat glands on a spectrum that involves both eccrine and apoeccrine lesions.

Apocrine cystadenoma and apocrine hidrocystoma: examination of 21 cases with emphasis on nomenclature according to proliferative features.

BACKGROUND: Apocrine cystadenoma (AC) and apocrine hidrocystoma (AH) have been used interchangeably in the literature to designate cystic lesions of apocrine glands. METHODS: We reviewed 21 cases with biopsies of apocrine cystic lesions diagnosed as AH or AC stained by hematoxylin and eosin. The following histological characteristics were recorded: (a) number of cysts, (b) predominant architectural growth pattern of cyst wall, (c) tumor circumscription, (d) nuclear atypia, (e) mitotic activity, counted per 1 mm2 and (f) Ki-67 staining pattern. RESULTS: Our findings clearly show that there is a non-proliferative group and a proliferative group among the lesions. In the non-proliferative group, one may see some structures that resemble papillary projections but lack a fibrous core. In the proliferative group, we found true papillae, and this change was associated with atypia, mitotic activity and increased Ki-67 staining. CONCLUSIONS: Apocrine cystic lesions with true papillary projections should be referred to as AC rather than AH, to emphasize the proliferative adenomatous growth and depicted by their frequency of cytological atypia and high mitotic activity. Furthermore, we suggest complete excision of AC that are proliferative tumors.

Tubular adenoma and syringocystadenoma papilliferum: a reappraisal of their relationship. An interobserver study of a series, by a panel of dermatopathologists.

Tubular adenoma (TA) and syringocystadenoma papilliferum (SCAP) may show histopathological overlap, with some lesions having features of both neoplasms (SCAP + TA). TA has been recently suggested to represent a carcinoma. Four observers blindly assessed 67 cases of TA, SCAP, and their lookalikes (poroma, apocrine adenoma, apocrine carcinoma; all lesions focally featuring a pseudopapillary pattern), and classified the lesions into one of four categories: (1) TA, (2) SCAP, (3) SCAP + TA, and (4) others. Lesions were also classified as benign or malignant. In only 29 cases was there unanimous agreement among the four observers, who classified 22 lesions as TA, three as SCAP, and four cases as others. Of the 38 cases where there was interobserver diagnostic variation, in 30, the diagnosis varied between TA or SCAP or SCAP + TA; the remainder fell in the others category. Analysis of the factors leading to interobserver variability indicated that diagnostic problems occurred when there were any of the following: epidermal acanthosis, papillomatosis, connection of the neoplastic tubules to the overlying epidermis and/or follicular infundibula, and plasma cell infiltration. These features accounted for the morphological overlap between TA and SCAP. All observers agreed that the lesions were benign; the only apocrine carcinoma included was recognized as such by all observers. From the study, it was concluded that TA may arise in the deep dermis without any epidermal connection, or, in other cases, it may be more superficially located with or without an epidermal connection. It may be reasonably inferred that, possibly as a response to infection, there may be accompanying plasma cells and variable acanthosis and papillomatosis, such that the appearances are those of "pure" SCAP, or lesions may have features "intermediate" or overlapping between TA and SCAP.

High (nuclear) grade adnexal carcinoma with microcystic adnexal carcinoma-like structural features.

Microcystic adnexal carcinoma (MAC) is a slow growing, locally aggressive sweat gland tumor. It predominantly affects the face and tends to recur despite local excision. Microscopically, MAC is characterized by a stratified proliferation of microcysts, cords, and ducts of cells that show squamous or adnexal differentiation. Atypia and mitoses are almost completely absent and metastatic deposits are rare and mostly limited to the regional lymph nodes; rather than real metastases, they might be the result of local extension of the tumor through perineurial spaces. We report a case of adnexal carcinoma with architectural features of MAC that displayed also marked nuclear pleomorphism and hyperchromasia with squamous pearl formation and a widespread strong p53 immunoreaction. The lesion behaved as a high-grade neoplasm with rapid growth, carcinosarcomatous metaplastic transformation in a relapse, and what were clinically suspected to be metastases. The literature contains several other examples reported as metastatic high-grade MAC, one of them with widespread distant metastases. We therefore want to sound an alert about the possible existence of tumors displaying microscopic findings characteristic of the aggressive forms of sweat gland carcinoma (nuclear pleomorphism and hyperchromasia, vascular invasion, and necrosis) in addition to architectural features of MAC. Whether these tumors should be called high-grade MACs or belong to a separate category remains an open issue until more cases are reported and bridge cases are eventually documented.

Tubular apocrine adenoma in association with syringocystadenoma papilliferum.

Tubular apocrine adenoma is a very rare sweat gland tumor. In this report, a case of tubular apocrine adenoma in association with syringocystadenoma papilliferum on the scalp is presented. The stroma of the tubular apocrine adenoma consisted of numerous, young fibroblasts with mitotic activity. It was difficult to distinguish stromal cells and epithelial cells from each other in some areas. The characteristics and differences in histopathologic and immunohistochemical findings in these tumors are described.