Pure apocrine nevus. A study of light-microscopic and immunohistochemical features of a rare tumor

The pure apocrine nevus is a rare tumor. A new case of pure apocrine nevi of the bilateral axillary regions in a 48-year-old man is herein reported. The patient had two soft masses of both axillae. The time of onset of the masses was unknown. They were not accompanied by problems of sweating. Histologically, these lesions comprised numerous apocrine glands extending from the reticular layer of the dermis to the subcutaneous tissue. In addition, sections from both of the lesions were stained by immunoperoxidase techniques for the presence of epithelial membrane antigen, carcinoembryonic antigen, and the alpha and beta subunits of S100 protein. Immunohistochemical features of the tumor stained by these antibodies are consistent with a pure proliferation of mature, well-differentiated apocrine glands. The tumor of the present case seems to have developed spontaneously. It is believed, therefore, that the tumor seen in this patient is best categorized as a pure apocrine nevus.     

Congenital Apocrine Hamartoma: An Unusual Clinical Variant of Organoid Nevus with Apocrine Differentiation

Abstract: Congenital apocrine hamartomas are rare tumors of the skin composed of mature apocrine glands in the papillary and reticular dermis. Several cases have been reported in the literature but few were of uniform clinical appearance. The term apocrine nevus has been used interchangeably with apocrine hamartoma in the literature, however, based on their distinctive morphologies we believe they are different entities. We describe a 7-year-old girl with a congenital apocrine hamartoma of the left cheek. A stained biopsy specimen from the lesion revealed large mature apocrine glands with decapitation secretion in the dermis. The presence of periodic acid-Schiff-posltive, diastase-resistant granular material in the apical cytoplasm of some secretory cells helped differentiate these as apocrine glands. We think this lesion may represent a form of organoid nevus with pure apocrine differentiation.     

Ductal carcinoma arising from a syringocystadenoma papilliferum in a nevus sebaceus of Jadassohn

We present an example of ductal carcinoma connected to a syringocystadenoma papilliferum situated in a nevus sebaceus of Jadassohn on the scalp of a 22-year-old woman. The ductal carcinoma involved the entire thickness of the dermis and extended to the subcutaneous fat. Because syringocystadenoma papilliferum is considered a hamartoma with apocrine differentiation, the ductal carcinoma here described was interpreted as an apocrine ductal carcinoma. Syringocystadenocarcinoma papilliferum is an exceedingly rare neoplasm, most examples of which seem to have arisen in its benign counterpart, syringocystadenoma papilliferum. From a histopathologic point of view, syringocystadenocarcinoma papilliferum usually shows a papillary configuration similar to that of syringocystadenoma papilliferum. In contrast, the case here described a ductal carcinoma superficially connected to a syringocystadenoma papilliferum, but mostly composed of small ductal structures embedded in a desmoplastic stroma and involving the full thickness of the dermis. We review the literature about the malignant neoplasms arising in the nevus sebaceus of Jadassohn.     

皮脂腺痣并发乳头状汗管囊腺瘤及管状乳头状顶泌汗腺腺瘤

报告1例皮脂腺痣并发乳头状汗管囊腺瘤及管状乳头状顶泌汗腺腺瘤.患者女,26岁.因右侧头皮黄色无毛斑块26年就诊.体格检查示右侧头皮黄色无毛斑块,边界清楚,表面粗糙,局部糜烂渗液.组织病理检查示表皮疣状增生,皮脂腺和顶泌汗腺增生.一部分顶泌汗腺与表皮相连,形成乳头状突起,其间质中有较多浆细胞浸润;另一部分顶泌汗腺位于真皮深部,不与表皮相连,形成较多管腔样结构,管腔内见顶泌汗腺呈乳头状突入管腔.结合临床资料及组织病理学改变,本病为1例罕见的皮脂腺痣并发乳头状汗管囊腺瘤及管状乳头状顶泌汗腺腺瘤.     

Axillary apocrine carcinoma associated with apocrine adenoma and apocrine gland hyperplasia

Apocrine carcinomas represent a rare group of tumors with a potential for destructive local invasion, regional and distant metastases, and are equally common in both sexes. A case of a 79-year-old woman with axillary apocrine carcinoma associated with apocrine adenoma and apocrine gland hyperplasia is presented. To our knowledge, this is the first case diagnosed in a Caucasian and also the first case diagnosed in a female patient. Grossly, the tumor measured 3.2x1.5x1.2 cm and on cut section appeared granular, white to gray-tanned. Microscopically, the tumor was located in the dermis, poorly demarcated, focally necrotic with ulcerated overlying skin. It was predominantly composed of complex, closely packed tubuloglandular structures but in few areas papillary structures were also observed. The cells contained abundant eosinophilic, finely granular cytoplasm with pleomorphic nuclei and showed apocrine-like decapitation. The cytoplasm contained periodic acid Schiff diastase resistant granules. Mitoses were frequent and some were atypical. In one area, the tumor was lobular and composed of tubular structures lined with one layer of uniform cuboidal or columnar eosinophilic cells, indicating a pre-existing apocrine adenoma. Beneath the tumor, in the deep dermis and subcutaneous tissue, hyperplastic apocrine glands were also found. No additional therapy was used, and one year after the surgery the patient was alive and showed no signs of tumor spread. This and previously reported cases suggest that apocrine hyperplasia and apocrine adenoma may represent successive steps in the development of apocrine carcinoma.     

Apocrine poroma: a distinctive case in a patient with nevoid basal cell carcinoma syndrome

Traditionally, poromas have been classified as eccrine neoplasms, but several recent reports of poroid tumors with sebaceous, follicular, and apocrine differentiation have challenged this idea. In support of alternative differentiation, a case of an "apocrine" poroma is reported in a 19-year-old man with the nevoid basal cell carcinoma syndrome. A papule on the right cheek, thought clinically to be a basal cell carcinoma, was excised. Anastomosing lobules of small uniform basaloid (poroid) cells formed small ductular structures lined by eosinophilic cuticles and extended into the superficial reticular dermis. The neoplasm originated from follicular infundibula and was surrounded by a myxoid stroma. Focally, primitive hair bulb and papillae differentiation was present, and some of the ducts were lined by cells suggesting decapitation secretion. The histologic pattern and the common embryologic origin of the folliculosebaceous-apocrine unit support apocrine differentiation of this tumor. The association with the nevoid basal carcinoma syndrome appears to be unique. This case, in addition, demonstrates overlapping features with the infundibulocystic type of basal cell carcinoma commonly seen in the basal cell nevus syndrome.     

Hidradenoma papilliferum with mixed histopathologic features of syringocystadenoma papilliferum and anogenital mammary-like glands: report of a case and review of the literature

Hidradenoma papilliferum of the anogenital region was previously believed to originate from apocrine glands but has recently been accepted as originating from anogenital mammary-like glands. We describe a case of hidradenoma papilliferum with mixed features of syringocystadenoma papilliferum and mammary-like glands from the left labia majora of a 25-year-old woman. Histopathologically, the lesion showed an epithelial lining with apocrine secretion, and like syringocystadenoma papilliferum, the lesion extended from the epithelium as invaginations into the dermis. Adjacent to this lesion were ductal and glandular structures resembling normal mammary tissue. This review of the literature highlights the heterogeneity and complexity of lesions arising from anogenital mammary-like glands, and this case serves as further documentation of the association between anogenital mammary-like glands and hidradenoma papilliferum.     

Low-grade adnexal carcinoma of the skin with multidirectional (glandular, trichoblastomatous, spiradenocylindromatous) differentiation

The conjoint occurrence of follicular, sebaceous, or apocrine differentiations in a cutaneous adnexal neoplasm is a known event, more often encountered in benign neoplasms, whereas reports of cutaneous malignant adnexal tumors with bilineage or trilineage differentiation are few. A new case of a cutaneous malignant adnexal neoplasm with multidirectional differentiation is reported here. A 57-year-old woman presented with a long-standing, slowly growing, asymptomatic solitary tumor the size of a large nut in the coccygeal area, which was surgically excised. Ten years after the surgery, there was no evidence of recurrence or metastasis. Microscopically, the neoplasm was located in the dermis with focal extension into the subcutis. It was asymmetric, horizontally oriented, and mostly composed of small nodules that varied in shape from round and oval aggregations to elongated strands and irregular islands; the nodules were either clustered, formed a jigsaw puzzle-like pattern or were dispersed. The nodules were composed of small basaloid cells sometimes intermixed with larger cells with ample cytoplasm forming glandular structures. Rare nodules resembled elements seen in a spiradenoma by containing scattered lymphocytes and globules of hyalinized eosinophilic basal membrane material. The stroma was paucicellular, but focally it resembled that seen in perifollicular mesenchyme. Mitotic figures, including abnormal ones, were infrequent, but mild nuclear pleomorphism, nuclear crowding, and individual cell necrosis were easily appreciable in both small basaloid cells and cells with clear cytoplasm. Perineural invasion was apparent. We classified this tumor as a well-differentiated adnexal carcinoma demonstrating combined follicular and apocrine differentiation. It differs from previously published cases of malignant adnexal tumors with multidirectional differentiation and further exemplifies the spectrum of diversity encountered in malignant proliferations with differentiation toward the folliculosebaceous-apocrine unit.     

Sebaceous carcinoma with apocrine differentiation

A 54-year-old male had a dome-shaped and skin-colored nodule on his nose. Histopathologically, we diagnosed this neoplasm as a low-grade sebaceous carcinoma rather than a sebaceoma based on the scanning magnification and cytology. This low-grade sebaceous carcinoma was associated with glandular structures. We regarded the glandular structures as those of apocrine glandular differentiation based on 1) the histopathologic features of the glandular structures formed by columnar luminal cells with evidence of decapitation secretion; 2) the expression of cytokeratin (CK) 19, CK8, CK8/18, and CK7 in the luminal cells; 3) the positive reaction of carcinoembryonic antigen and epithelial membrane antigen on the luminal surface and in the cytoplasm of the luminal cells; and 4) the common embryologic origin of the folliculosebaceous-apocrine unit. We found CK15 expression in undifferentiated cells within the mantles of normal hair follicles, suggesting that sebaceous stem cells might exist in mantles as follicular stem cells exist in bulge areas. Pluripotent stem cells in the folliculosebaceous-apocrine unit can give rise to follicular stem cells, sebaceous stem cells, and apocrine stem cells. Our patient's neoplasm showed apocrine glandular differentiation and partial immunohistochemical positivity for CK15 in the neoplastic aggregations. We believe this neoplasm originated from pluripotent stem cells destined to become sebaceous stem cells or from sebaceous stem cells, which also have the ability to differentiate within apocrine glands.     

Apocrine nevus: light microscopic, immunohistochemical and ultrastructural studies of a case

The apocrine nevus (AN) is a rare tumor occurring in the upper chest and the axilla. We report a case of a AN in a 33-year-old female occurring unilaterally. The presenting complaint related to tenderness and swelling in the right axilla. The initial impression was hidradenitis suppurativa. The gross specimen revealed the presence of irregular thickening just beneath the dermal subcutaneous interface. Microscopically the lesion was composed of mature apocrine glands with apical snouts. The glands were arranged in lobules divided by thin fibrous septa. immunohistochemical studies revealed the following profile in the glandular epithelium: positive low molecular weight cytokeratin, epithelial membrane antigen, and gross cystic disease fluid protein reactivity and negative high molecular cytokeratin and S-100 protein reactivity. Carcinoembryonic antigen reactivity was found in the duet epithelium. Ultrastructural studies revealed cells lining the lumen of the glands with a concentration of granules in the apical region and light and dark granules. These findings support the previously described light microscopic observations and provide unreported ultrastructural studies in this rare tumor.     

腋臭患者腋区顶泌汗腺的分布特点

【摘要】 目的 检测腋臭患者腋区顶泌汗腺在深度、广度的分布,探讨其范围内的分布差异性。 方法 2010年9 - 12月间的15例腋臭患者,行直视下顶泌汗腺剪除术,切取切口处宽约2 mm的全层皮肤,深度达腋浅筋膜浅层,用于判明顶泌汗腺分布的深度。留取腋中心（点1）、距腋中心1 cm（点2）、距腋毛边缘内1 cm（点3）、腋毛边缘（点4）、距腋毛边缘外1 cm（点5）共5个标记点对应的皮下暗红色粗大的颗粒状组织,用于判明顶泌汗腺的分布范围及分布规律。 结果 顶泌汗腺分泌部主要分布于真皮网状层和皮下脂肪浅层,表皮层、真皮乳头层及腋浅筋膜浅层均无顶泌汗腺分布。顶泌汗腺广度分布与腋毛范围基本一致,腋中心部可见大量顶泌汗腺组织,腋毛边缘处仍有少量顶泌汗腺分布,腋毛边缘外1.0 cm处已无顶泌汗腺分布。5个点顶泌汗腺面积占整块组织面积的百分比平均值分别为74.1%、46.6%、25.3%、2.1%和0,相邻两点进行t检验,点1、2之间t = 29.78,P < 0.01;点2、3之间t = 9.76,P < 0.01;点3、4之间t = 20.83,P < 0.01;点4、5之间t = 1.96,P > 0.05。 结论 手术治疗腋臭时,清除范围深度达真皮网状层和浅层脂肪层,广度到腋毛范围即可,没有必要过度扩大清除范围。     

Malignant transformation of a nevus of Ito: description of a rare case

Dermal melanocytosis refers to congenital or acquired lesions characterized by the presence of dendritic cells derived from melanocytes that migrate from the neural crest to the epidermis. The nevus of Ito develops in the territory supplied by the acromioclavicular nerve. Malignant transformation in dermal melanocytosis is extremely rare, with only isolated case reports; only 2 cases of malignant transformation of a nevus of Ito have been reported. We report a very rare case that is the third to be described in the literature. The patient was a 24-year-old man who presented with a subcutaneous nodule that had developed in the anterolateral region of the thorax over the previous 8 months. The nodule was located beneath a faint blue-gray macule with poorly defined borders. Biopsy of the nodule revealed malignant melanoma; biopsies of the adjacent skin lesion showed a diffuse proliferation of scattered melanocytes in a collagen stroma in the reticular dermis. A diagnosis of malignant transformation of a nevus of Ito was made after other possibilities were ruled out.     

Cutaneous endometriosis: diagnostic immunohistochemistry and clinicopathologic correlation

Endometriosis is a condition where endometrial glands and stroma are ectopically located in sites other than the uterine cavity. Cutaneous endometriosis is very rare, representing approximately 1.1% of cases of extrapelvic endometriosis. We report a case of a 44-year-old female with no prior surgical history who presented with multiple tan brown periumbilical nodules. Histopathological examination revealed multiple glandular structures in the dermis with surrounding stroma. Immunohistochemistry cinches the diagnosis, as CD10, estrogen receptor and progesterone receptor are strongly positive in our case. The mainstay of treatment of cutaneous endometriosis is surgical excision of the lesion.     

Buschke‐Ollendorff syndrome with LEMD3 germline stopgain mutation p.R678* presenting as multiple subcutaneous nodules with mucin deposition

Buschke‐Ollendorff syndrome (BOS; OMIM 166700) is a rare autosomal dominant disorder characterized by the existence of connective tissue nevus and/or osteopoikilosis. The skin lesions usually present as firm, yellow, or flesh‐colored papules and nodules, which may coalesce into plaques and increase in size and number over time. We present a case of a 26‐year‐old male with multiple subcutaneous nodules on the waist and thigh for more than 20 years. Being deeply seated, his skin lesions were not visible and could only be appreciated by palpation. Accordingly, pathology showed an increase in thick, crossed, or paralleled, elastic fibers arranged between the collagen bundles in the lower part of the reticular dermis and the subcutaneous fat with mucin deposition. Heterozygous point mutation in exon 8 of the LEMD3 gene was detected, which confirmed the diagnosis of BOS. The deeply situated nature of skin lesions noted in our case has not been reported in the literature of BOS. Our case thus expands the clinical and pathological features of the disease.     

Desmoplastic fibroblastoma

Previous reports of a distinctive, fibrous, soft-tissue tumor include eight patients with subcutaneous lesions and six patients with intramuscular lesions. We report a 48-year-old woman with a 2-cm cutaneous and subcutaneous nodule on the left arm with the same histologic features. An excisional biopsy showed a large, well circumscribed tumor replacing the reticular dermis and subcutaneous tissue. The tumor was relatively hypocellular and composed primarily of large, spindled, plump or stellate fibroblasts haphazardly dissecting between thickened Fibrotic collagen bundles. The stroma contained a large amount of much which was positive with alcian blue at pH 2.5, and relatively numerous mast cells were present. The fibroblastic-like cells were positive with Vimentin and Factor XIIIA and negative with S-100, desmin, actin and keratin.     

Histopathology of congenital pigmented nevi

Between 1982 and 1985, a total of 39 congenital pigmented nevi (CN) were removed from patients in the Oklahoma Children's Memorial Hospital. Multiple sections were examined from each of the lesions removed. The histologic patterns were recorded with special note of those features traditionally associated with CN. These include nevus cells in the lower reticular dermis and subcutaneous tissue, association of nevus cells with and in appendageal structures, and infiltration of nevus cells between collagen bundles. These features were primarily observed in larger CN (greater than 3 cm). Most small nevi do not show these features. The histologic patterns were consistent throughout multiple sections of the nevi. It is concluded that the histology traditionally associated with CN relates primarily to the size of the nevus rather than to the time of its appearance.     

Mucinous hidradenoma: a report of three cases

Mucinous cutaneous adnexal tumors are an uncommon occurrence. We describe three cases of mucinous hidradenoma characterized by diffuse and prominent mucinous cell proliferation and discuss the relevant literature. The patients (two men and one woman) ranged in age from 32 to 72 years. The lesions were completely excised, and all patients were alive without evidence of disease (follow-up interval 3-4 years). Case 1 presented as a 1.3-cm eyelid nodule and histology showed a mucinous apocrine hidradenoma with a proliferation of mucinous cells and apocrine glands. Case 2, from the shoulder of a 40-year-old man, was a 2.5-cm well-circumscribed nodule composed of hyperplastic villoglandular proliferation of mucinous cells with focal squamous whorls and focal columnar glandular cells showing decapitation secretion. The tumor was also interpretated as a mucinous hidradenoma, most likely of follicular-apocrine origin. Case 3 was a 3.0-cm scalp nodule. The histology showed a mucinous clear-cell hidradenoma composed of numerous mucinous glands amidst small squamous cells with clear cytoplasm and a hyalinized fibrovascular stroma.     

Squamous cell apocrine hidradenoma

Apocrine hidradenoma is a benign adnexal neoplasm with apocrine differentiation. The neoplasm is composed of four different types of epithelial cells, including pale or clear cells, polygonal cells, mucinous cells and squamous cells, with variable proportions of them from case to case. In most examples of this neoplasm, clear or the polygonal cells are predominant, whereas the other types of neoplastic cells are less abundant. We report two cases of apocrine hidradenoma mostly composed of squamous cells. Histopathologic examination showed that the neoplasms were composed of both solid and cystic areas. The solid aggregations of neoplastic cells were composed of a peripheral layer of basaloid polygonal cells, whereas squamous cells forming the bulk of the aggregations. These squamous cells showed large eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli. In one case, small foci of mucinous cells could also be seen in some aggregations of neoplastic cells, mostly around ductal structures. In both the cases, some of the tubular structures lined by epithelial cells showed evidence of decapitation secretion in their luminal border. The neoplastic stroma consisted of sclerotic collagen bundles when compared with adjacent normal dermis, and artefactual clefts separated the neoplasms from the surrounding tissue. The rare cases described in this report are exceptional because most of the neoplastic cells showed squamous appearance and for that reason we think that squamous cell apocrine hidradenoma is the most appropriate name for these neoplasms.     

An unusual melanocytic lesion associated with eccrine duct fibroadenomatosis and syringoid features

The intimate association of nevomelanocytic nevi with eccrine ducts commonly seen in congenital nevi was emphasized by Mishima, who described as eccrine-centered nevi those lesions characterized by nevomelanocytic cells predominantly proliferating around and within the eccrine sweat duct walls. However, there were no changes in the overlying epidermis, dermis, or eccrine acrosyringeal or dermal duct proliferation in these lesions. We present the case of a 16-year-old boy with a 1-year-history of a 0.6-cm diameter single tan papule on the right heel, clinically thought to be a Spitz nevus. Histopathologic examination revealed a compound nevomelanocytic nevus associated with epidermal hyperplasia, thin anastomosing cords of acrosyringeal epithelium extending within the dermis, and eccrine ductal proliferation in a syringoma-like pattern associated with a dense fibrous stroma. Features that distinguish our case from eccrine-centered nevus are that the latter lacks epidermal and eccrine duct hyperplasia and a dense fibrous stroma. The location of the lesion on the heel in our case suggests the possibility that the pathologic changes observed could result from repetitive trauma.