Apocrine cystadenoma: A long-standing apocrine hidrocystoma with an adenomatous proliferation.

BACKGROUND: Apocrine cystadenoma is a rare, benign adenomatous cystic neoplasm, the pathogenesis of which is not fully understood. We sought to characterize the clinical, dermatoscopic, and histopathologic features of apocrine cystadenoma and its relationship to hidrocystoma. METHODS: We retrospectively analyzed cases of apocrine cystadenoma and hidrocystoma retrieved from the dermatopathology laboratory information system. RESULTS: Of the 350 cases apocrine cystic lesions, 13 cases of apocrine cystadenomas met the inclusion criteria. The age ranged from 20 to 84 years with an average of 64 years. They were long-standing (duration 3-15 years), slow-growing, large tumors usually found on the scalp. Dermatoscopy accentuated translucent light to dark blue color and prominent vessels that were present more at the periphery. All lesions were multilocular with columnar to cuboidal lining and decapitation secretion. A large portion of the lesion consisted of a simple nonproliferative epithelial lining, identical to that observed in apocrine hidrocystomas, while the proliferative adenomatous component made up a smaller portion with two patterns: (1) tubular proliferation, which either protruded into the cystic cavity or expanded outward peripherally, or (2) papillary projections, which were multiple layers thick with fibrovascular core, sometimes accompanied by tubular proliferation. Immunohistochemical stains showed strong staining for p40 and a sparse number of cells stained for Ki-67 and p53. CONCLUSIONS: The long duration of the lesion and the large areas of simple apocrine epithelial lining suggest that apocrine cystadenomas arise from long-standing apocrine hidrocystomas. However, the retrospective nature of the study from a single institution is a limitation.

Primary cutaneous apocrine carcinoma with RARA::NPEPPS fusion.

Gene fusions have emerged as crucial molecular drivers of oncogenesis in a subset of cutaneous adnexal neoplasms, including poroid neoplasms and hidradenomas. We present a unique case of primary cutaneous apocrine carcinoma harboring RARA::NPEPPS fusion, broadening the spectrum of fusion-associated cutaneous adnexal neoplasms. A 77-year-old African American male presented with an ulcerated thigh nodule. Histopathologically, the predominantly dermal-based adenocarcinoma exhibited papillary, micropapillary, cribriform, and solid growth patterns with central comedonecrosis, set in a fibrotic/desmoplastic stroma. Immunophenotypically, the neoplastic cells were positive for CK7, CK19, GATA3, TRPS1, HER2, CK5/6, calretinin, p63, and DPC4 (no loss), while lacking immunoreactivity for CK20, CDX2, TTF1, napsin-A, PAX8, arginase-1, adipophilin, NKX3.1, uroplakin II, and D2-40. The immunoprofile and clinical and radiographic absence of any internal malignancy, including breast carcinoma, except for multiple lymphadenopathy, supported the diagnosis of primary cutaneous apocrine carcinoma. Next-generation sequencing unveiled the novel RARA::NPEPPS fusion, concurrent ERBB2 amplification, and multiple somatic mutations involving TP53, CDKN2A, BRCA2, PIK3CA, PIK3R1, and others. The patient developed widespread metastases within a year after the initial diagnosis, indicating the tumor's aggressive behavior. This novel fusion, unprecedented in any human malignancies including primary cutaneous adnexal carcinomas, may suggest a potential new subtype within primary cutaneous adnexal carcinoma.

Hidradenitis suppurativa disease control associated with type of hormonal contraceptive use.

Hidradenitis suppurativa (HS) is a painful chronic skin condition of apocrine gland regions. This retrospective cohort study aimed to assess the impact of hormonal contraception type on HS disease control in adult women. In total, 160 patients were included, with the majority identifying as Black or African American (73.1%). Multivariate logistic regression showed that oestrogen-progesterone users were 3.14 times more likely to experience stable or improved HS than progesterone-only users (adjusted odds ratio 3.14, 95% CI 1.18-8.35; P = 0.02). Further investigation is needed to elucidate the antiandrogenic mechanisms affecting HS symptom response to hormonal contraceptives.

Apocrine Hidrocystoma of the Nail: A Unique Case.

ABSTRACT: Apocrine hidrocystomas are benign, cystic neoplastic lesions resulting from the apocrine secretory component of the sweat gland. They most commonly occur on the head and neck, with predilection to the periorbital area. Less frequent sites include the axilla, nipple, external auditory canal, foreskin, conjunctiva, lower lip, and fingers, among others. The authors report a unique case of a nail bed hidrocystoma in a 55-year-old woman, a site not previously described.

Immunohistochemical Expression of Human Epidermal Growth Factor Receptor 2 and Ki67 in Apocrine Gland Anal Sac Adenocarcinoma.

Apocrine gland anal sac adenocarcinoma is an aggressive neoplasm, and surgery remains the treatment of choice, although it is controversial in advanced cases. The prognostic factors are not well established. Human Epidermal Growth Factor Receptor 2 (HER2) is a membrane protein related to tumorigenesis, whereas Ki67 is a nuclear protein related to cell proliferation. Both are potential prognostic markers and therapeutic targets. This study aimed to evaluate the expression of HER2 and Ki67 markers in canine apocrine gland anal sac adenocarcinoma. The tumor samples were divided into four groups: largest tumor diameter less than 2.5 cm, largest tumor diameter greater than 2.5 cm, metastatic lymph nodes, and control group of non-neoplastic anal sacs. Each contained 10 samples. Immunohistochemistry was performed to verify the expression of HER2 and Ki67 markers. Positive HER2 staining was observed in 45% of the neoplastic cases and negative HER2 staining in 100% of the control group. The Ki67 expression had a median of 25% in all groups, except for the control group, which had a median of 8%. The HER2 and Ki67 expression was present in apocrine gland anal sac adenocarcinoma, making them potential therapeutic targets. However, it was not possible to determine the clinical value of either marker.

Secretory Carcinoma of the Breast with Apocrine Differentiation-A Peculiar Entity.

Background and Objectives: Secretory carcinoma of the breast is an uncommon histological subtype of breast cancer. There is little research on this entity and only a few larger studies, which lack consensus. We aim to report a particular apocrine differentiation in this subtype and ponder upon the clinical outcome of this case. Case presentation: We report the case of a 72-year-old female patient who presented to our hospital with a suspicious breast tumor. Core biopsy and mastectomy showed a low-grade breast carcinoma, a secretory subtype with apocrine differentiation. Immunohistochemistry confirmed both the secretory nature and the apocrine nature of the tumor cells. Surgical excision was considered curative and the patient is under long-term surveillance for any recurrences. Conclusions: There is very little research on the clinical behavior of secretory carcinomas with apocrine differentiation. The clinical outcome is unknown and, unfortunately, besides surgery, no other adjuvant treatments have shown efficacy. Further studies on long-term clinical progression are required for this rare entity.

Primary cutaneous apocrine carcinoma, arising in tubular apocrine adenoma.

Primary cutaneous apocrine carcinoma is a rare adnexal tumor that arises from apocrine progenitor cells. These tumors may be associated with benign apocrine hyperplasia, and a longstanding history of a lesion should not preclude a malignant diagnosis. We report a case of a 70-year-old female who presented to the clinic with a 3-year history of an asymptomatic vulvar lesion. An excisional biopsy was performed. Histopathologic examination revealed a tumor with two distinct components. The first component was determined to be a benign tubular apocrine adenoma. The second component, arising within the apocrine adenoma, was determined to be an apocrine carcinoma based on histopathologic features and immunohistochemical profile. Twelve months after subsequent wide local excision and sentinel node biopsy, the patient is alive without recurrence.

Morphological study of apocrine gland tumors in domestic Richardson's ground squirrels (Urocitellus richardsonii).

The morphology of 58 apocrine tumors from 31 domestic Richardson's ground squirrels (Urocitellus richardsonii) was examined, comparing tumor subtypes with clinical information and histological findings. The squirrels included 23 males (74%) and 8 females (26%). Of the 58 tumors, 36 (62%) were located from the cervical skin to the dorsal skin, 17 (29%) were from the perioral region to the cheek, and the location was unrecorded for the remaining 5 squirrels (9%). Histologically, 49 tumors (84%) were apocrine adenocarcinomas, 6 were apocrine adenomas (10%), and 3 were apocrine cysts (5%). The 49 apocrine adenocarcinomas were subclassified as 16 (33%) cystic papillary type, 16 (33%) tubulopapillary type, 12 (24%) solid type, and 5 (10%) micropapillary invasive type. Fisher's exact test revealed correlations between tumor subtypes and tumor diameters, nuclear atypia, tumor margins, vascular invasion, and periodic acid-Schiff (PAS) positivity. This study showed a predominance of apocrine adenocarcinomas with various histological subtypes. In addition, subclassification of these tumors was useful for predicting the biological behavior of apocrine gland tumors in Richardson's ground squirrels.

Apocrine Papillary Hidrocystoma With Mucinous Metaplasia (Goblet Cell Type): A Case Report and Review of the Literature.

ABSTRACT: Mucinous metaplasia (goblet cell type) is exceptionally rare in the skin. This is the second case of apocrine papillary hidrocystoma with mucinous metaplasia (goblet cell type) and a review of the literature exploring the significance and frequency of mucinous metaplasia with goblet cells in nongenital skin. The patient is an elderly man who presented with a blue-pigmented nodule on the scalp that was clinically suggestive of an atypical nevus. Histologically, the lesion was composed of a simple cyst of cuboidal cells with decapitation secretion and mucinous metaplasia with goblet cells. Papillary formation was identified in the cysts. Most cases of cutaneous mucinous metaplasia have been reported on genital skin, usually after chronic inflammation of the area. This type of mucinous metaplasia is categorized as benign mucinous metaplasia of the genitalia (BMM) and is believed to be unrelated to apocrine glands owing to the different histologic features and absence of apocrine differentiation by immunohistochemistry. Mucinous metaplasia (goblet cell type) has been previously reported in benign adnexal tumors (eccrine acrospiroma/hidroadenoma, mixed tumor, and syringocystadenoma papilliferum) and in malignant tumors (apocrine hidradenocarcinoma and squamous cell carcinoma). To date, mucinous metaplasia has not been identified in the histologically normal apocrine glands.

Apocrine Cystadenoma of the Eyelid, a Rare Neoplasm: Expanded Immunohistologic Profile.

An 83-year-old woman experienced the slow enlargement of a right lower eyelid mass. Histopathologic examination of the excised tissue showed a mucin-filled cystic tumor emanating from an apocrine bilayer that displayed bleb-like apocrine decapitation secretion. The outer flattened myoepithelial layer of the bilayer reacted with immunohistochemical stains for smooth muscle actin and calponin. In foci, the tumor exhibited a cribriform architecture with small pockets of mucin. Tumor cells were reactive for cytokeratin 7, Gross Cystic Disease Fluid Protein 15 (BRST-2), estrogen and progesterone receptors, androgen receptors, mammaglobin, epithelial membrane antigen, and GATA3. Ki67 showed a very low proliferation fraction. The lesion exemplifies the fourth instance of an eyelid apocrine cystadenoma in the literature.

Pigmented apocrine hamartoma: A distinct and uncommon pigmented lesion with dendritic melanocytes and appraisal of terminology.

A 20-year-old female presented to a gynecologist with an irregular, darkly pigmented, vulvar lesion. Histopathologic sections of a biopsy specimen showed cystically dilated glands with apical snouts, pigmented secretion, and numerous dendritic melanocytes. The lesion was diagnosed as a pigmented apocrine hamartoma of the vulva. We report the fifth case of this uncommonly encountered entity and discuss the conflicting terminology in the literature of this rare, pigmented lesion.

Power-assisted rotary cutter with negative pressure suction through small incision for axillary osmidrosis.

BACKGROUND: The glandular fluid secreted by apocrine sweat glands is decomposed by parasitic bacteria on the skin surface and releases a bad smell called axillary osmidrosis. OBJECTIVE: To evaluate the effect of power-assisted rotary cutter with negative pressure suction through small incision for axillary osmidrosis. METHODS: From January 2017 to January 2022, 647 patients with axillary osmidrosis underwent power-assisted rotary cutter with negative pressure suction through small incision surgery to treat for axillary osmidrosis. The operation time (min), intraoperative bleeding (ml) and recovery time (days) were counted. Three months after operation, the amount of underarm sweat, the disappearance of underarm hair, the incidence of operation, the effective rate of operation, and the satisfaction of operation results were evaluated by questionnaire. RESULTS: There were no postoperative complications except five cases of mild axillary pigmentation after 3 months. All patients evaluated good odor elimination. No patient was rated as average or poor. No recurrence. CONCLUSION: Power-assisted rotary cutter with negative pressure suction through small incision is an effective and ideal surgical method to reduce the complications and recurrence of axillary osmidrosis.

A Rare Case of Lesion in the Vulva: Pigmented Apocrine Hamartoma.

ABSTRACT: Apocrine hamartoma is a rare benign neoplasm. The histology is characterized by an excess of apocrine glands located predominantly in the reticular dermis. Pigmented apocrine hamartoma represents a histopathological variation of apocrine hamartoma containing tubules and linear cysts covered by apocrine cells on the inside with melanin and on the outside with myoepithelial cells. At this time, 4 cases of this pathology have been described. This case report aims to present a case of pigmented apocrine hamartoma of the vulva in a young patient, emphasizing that while occurrence is rare, it must be considered when diagnosing a pigmented lesion of the vulva in young patients.

Poorly Differentiated Scrotal Carcinoma With Apocrine Immunophenotype.

ABSTRACT: Cutaneous carcinoma of the scrotum is rare with the most common type being squamous cell carcinoma. Here, we report 6 cases of poorly differentiated carcinoma with apocrine immunophenotype. Mean age at presentation was 68 years (range: 31-91 years). Clinical presentation included eczematous rash over mass, scrotal cyst, ulcerated mass, and mass. Tumor size ranged from 1.2 to 5.5 cm (average 2.5 cm). The tumors were solid with involvement of the dermis/hypodermis and composed of cords and nests of eosinophilic cells displaying nuclei with prominent nucleoli and surrounded by desmoplastic stroma. Focal squamous differentiation was evident in one case (17%). An intraductal component was seen in one case (17%). Pagetoid spread in the epidermis was seen in 3 cases. There was no morphologic evidence of apocrine differentiation. By immunohistochemistry, the tumor cells were positive for GCDFP-15 (n = 6/6), GATA3 (n = 6/6), CK7 (n = 5/5), AR (n = 4/4), and mammaglobin (n = 3/5). Five (83%) patients had metastases at diagnosis. Treatment included wide local excisions and inguinal lymph node dissection, followed by chemotherapy (gemcitabine, carboplatin; n = 3), trastuzumab/Lupron (n = 1), tamoxifen/Arimidex (n = 1), and radiotherapy (n = 1). Two patients (40%) were dead of disease, less than 2 years from diagnosis. Four patients developed metastases to lymph nodes, liver, bones, and lungs. Molecular analysis (n = 2) detected a HER-2 mutation in one and microsatellite instability in another. Although the presence of an intraepidermal pagetoid component could hint toward the diagnosis of invasive extramammary Paget disease, tumors without an intraepidermal component could be diagnostically challenging given the lack of morphologic evidence of apocrine differentiation.

Ciliary Gland Adenocarcinoma of the Eyelid Arising in Hidrocystoma: Case Report and Review of Previously Reported Cases.

ABSTRACT: Moll gland is a modified tubular-shaped apocrine sweat gland, which is located on the margin of the eyelid. Moll glands are also known as ciliary glands. The function of these glands was not well known for a long time. However, based on the different studies, it was proved that Moll glands are active from birth and have local immunologic function producing immunoglobulin A. We present a case of Moll adenocarcinoma, a type of apocrine carcinoma, arising from the right upper eyelid of a 57-year-old man. Although Moll gland cysts are frequently seen, this type of adnexal malignancy is extremely uncommon. These tumors can have variable presentation and behavior from less invasive forms to highly malignant metastatic recurrent lesions.

Comparative Study of En Bloc Excision and Dermal Shaving in Treating Axillary Osmidrosis.

BACKGROUND: Axillary osmidrosis is a distressing problem caused by hyperactivity of apocrine glands. There have been numerous studies on various surgical treatment methods. In this study, we evaluated the effectiveness of en bloc excision in comparison with dermal shaving. METHODS: The electronic records of 146 patients (286 axillae) who underwent surgery at our center for axillary osmidrosis between January 2009 and December 2020 were reviewed. Twenty-five patients (49 axillae) underwent en bloc excision and 121 (237 axillae) underwent dermal shaving. Patients in the en bloc excision group underwent Minor test preoperatively to detect sweating areas. Severity of osmidrosis was graded using a 4-point scale (0-3). A satisfaction questionnaire was used to evaluate patient experiences in the 2 types. RESULTS: Mean operation time was significantly shorter in the en bloc excision group than in dermal shaving group. Most en bloc excisions were performed on an outpatient basis under local anesthesia. Both groups showed an improvement in osmidrosis score at 6 months after surgery. A satisfaction questionnaire revealed better perioperative experiences in the en bloc excision group. Various surgical complications such as hematoma, wound dehiscence, and flap necrosis occurred in the dermal shaving group, and the en bloc excision group experienced significantly fewer complications that required intervention. CONCLUSIONS: En bloc excision combined with Minor test effectively reduces malodor without causing severe complications. In addition, perioperative patient satisfaction was better in the en bloc excision group than in the dermal shaving group as en bloc excision provided more rapid returns to normality and simplified communications with patients.

Episcleral Apocrine Hidrocystoma Following Strabismus Surgery.

Apocrine hidrocystomas are benign cystic tumors derived from apocrine sweat glands; they are most commonly located in the skin of the head and neck regions. Ophthalmic occurrences typically appear at the lash line and canthi of the eyelid, although rare instances have been described in the conjunctiva, caruncle, and orbit. The authors illustrate an exceptional instance of a mobile episcleral cyst in a 12-year-old girl that developed about 2 years following strabismus surgery. The cyst was located anterior to the insertion of the left medial rectus and was excised in response to the patient's complaints of irritation. Histopathology of the excised specimen displayed an empty cyst lined by a double layer of cuboidal epithelium with the inner layer exhibiting apical decapitation secretion. Confirmatory immunohistochemistry demonstrated reactivity of both layers with CK7 and the outer myoepithelial layer with D2-40. Postoperative and traumatic cysts formed after interruption of the bulbar conjunctiva are usually conjunctival cysts lined by stratified squamous nonkeratinizing epithelium.

Minimal late radiation toxicity and transient early toxicity following postoperative definitive intent conformal radiation therapy (20 × 2.5 Gy) for canine apocrine gland anal sac adenocarcinoma.

Postoperative radiation therapy (RT) may be beneficial for dogs with anal sac apocrine gland adenocarcinoma (ASAC). Clinically significant late toxicities have been reported in up to 65% of dogs with perianal tumors following non-conformal definitive RT, particularly when fractions of 3 Gy or higher are prescribed. The primary objective of this prospective, descriptive study was to evaluate tolerability of a novel 3D conformal RT (3DCRT) protocol in a group of dogs. Dogs with ASAC were prospectively enrolled if clients elected RT following surgery. The planning target volume was prescribed 50 Gy in 2.5 Gy fractions using 6 MV photons and administered over 26 days. Early and late radiation toxicities were graded according to standardized criteria. Thirteen dogs were initially enrolled but 1 was excluded due to a high risk of anesthesia-related mortality. Seven dogs presented with early stage disease. Median follow up time was 771 days (91-2223). Transient grade 3 dermatitis and anusitis developed in all dogs, with resolution within 4 weeks. Two dogs developed transient grade 2 late colitis. Locoregional failure in the irradiated field was documented in one dog at 738 days. All-cause median survival time was 771 days (95% confidence interval: 510 â†’ 2223 days). Findings indicated that this fractionation may be safely administered to the canine anus and pelvic canal using 3DCRT, although acute toxicity should be anticipated. Further prospective studies are needed in order to confirm long-term tolerability and efficacy.

Complications associated with iliosacral lymphadenectomy in dogs with metastatic apocrine gland anal sac adenocarcinoma.

Objective: To report intraoperative and immediate postoperative complications associated with removal of metastatic iliosacral lymph nodes in dogs with apocrine gland anal sac adenocarcinoma. Animals: There were 136 client-owned dogs in the study. Procedure: Retrospective multi-institutional study. The database of collaborating institutions was searched for dogs with metastatic apocrine gland anal sac adenocarcinoma that underwent lymphadenectomy for removal of one or more iliosacral lymph nodes. Information of signalment, hematological abnormalities, abdominal computed tomography or ultrasound findings, number and size of enlarged lymph nodes, intraoperative and postoperative complications, treatment and outcome were collected. Results: The overall complication rate associated with metastatic iliosacral lymphadenectomy was 26.1%. The only intraoperative complication recorded was hemorrhage and was reported in 24 (17.6%) surgeries, 11 (45.8%) of which received a blood transfusion. Postoperative complications were reported in 10.4% of surgeries, and included edema formation (n = 4, 2.6%), unilateral or bilateral paraparesis (n = 4, 2.6%), hypotension (n = 3, 2.0%), surgical site infection (n = 2, 1.3%), abdominal incision dehiscence (n = 1, 0.6%), urinary incontinence (n = 1, 0.6%), and death (n = 1, 0.6%). The size of the iliosacral lymph nodes was significantly associated with a greater risk of complications, hemorrhage, and the need of transfusion during lymphadenectomy for metastatic apocrine gland anal sac adenocarcinoma. Conclusion: Complications associated with iliosacral lymphadenectomy for metastatic apocrine gland anal sac adenocarcinoma are relatively common and mostly relate to hemorrhage. These complications are significantly associated with the size of the extirpated metastatic lymph nodes. Clinical relevance: This retrospective study provides information for the clinician regarding the potential surgical complications for extirpation of metastatic iliosacral lymph nodes. These complications, although not common, can be severe and should be discussed with owners before surgery.

Objectif: Rapporter les complications peropératoires et postopératoires immédiates associées à l'ablation des ganglions lymphatiques ilio-sacrés métastatiques chez les chiens atteints d'un adénocarcinome des glandes apocrines des sacs anaux. Animaux: Il y avait 136 chiens appartenant à des clients dans l'étude. Procédure: Étude multi-institutionnelle rétrospective. La base de données des institutions collaboratrices a été recherchée pour les chiens atteints d'un adénocarcinome métastatique des glandes apocrines des sacs anaux qui ont subi une lymphadénectomie pour l'ablation d'un ou plusieurs ganglions lymphatiques ilio-sacrés. Des informations sur le signalement, les anomalies hématologiques, les résultats de la tomodensitométrie abdominale ou de l'échographie, le nombre et la taille des ganglions élargis, les complications peropératoires et postopératoires, le traitement et les résultats ont été recueillis. Résultats: Le taux global de complications associées à la lymphadénectomie ilio-sacrée métastatique était de 26,1 %. La seule complication peropératoire enregistrée était une hémorragie et a été rapportée dans 24 (17,6 %) chirurgies, dont 11 (45,8 %) ont reçu une transfusion sanguine. Des complications postopératoires ont été signalées dans 10,4 % des interventions chirurgicales et comprenaient la formation d'oedème (n = 4, 2,6 %), la paraparésie unilatérale ou bilatérale (n = 4, 2,6 %), l'hypotension (n = 3, 2,0 %), l'infection du site opératoire (n = 2, 1,3 %), la déhiscence de l'incision abdominale (n = 1, 0,6 %), l'incontinence urinaire (n = 1, 0,6 %) et le décès (n = 1, 0,6 %). La taille des ganglions ilio-sacrés était significativement associée à un risque accru de complications, d'hémorragie et à la nécessité d'une transfusion lors d'une lymphadénectomie pour un adénocarcinome métastatique des glandes apocrines des sacs anaux. Conclusion: Les complications associées à la lymphadénectomie ilio-sacrée pour l'adénocarcinome métastatique des glandes apocrines des sacs anaux sont relativement fréquentes et concernent principalement l'hémorragie. Ces complications sont significativement associées à la taille des ganglions lymphatiques métastatiques retirés. Pertinence clinique: Cette étude rétrospective fournit des informations au clinicien concernant les complications chirurgicales potentielles pour le retrait des ganglions lymphatiques ilio-sacrés métastatiques. Ces complications, bien que rares, peuvent être graves et doivent être discutées avec les propriétaires avant la chirurgie.(Traduit par Dr Serge Messier).

Atypical Non-neoplastic Changes in Anogenital Mammary-like Glands Accompanying Invasive Squamous Cell Carcinoma.

Long regarded as ectopic or supernumerary breast tissue, anogenital mammary-like glands (AGMLG) are now considered a normal constituent of the anogenital area. AGMLG are presumed to be the origin for various benign and malignant lesions. Changes in AGMLG compatible with usual ductal hyperplasia and atypical ductal hyperplasia considered as precursor lesions and its presence in specimens can be explained by their role in the pathogenesis of primary extramammary Paget disease. In this report, we presented four cases of invasive squamous cell carcinoma accompanied by non-neoplastic atypical changes in ductal portions of AGMLG compatible with atypical ductal hyperplasia in breast adjacent to the carcinoma. This is a reactive phenomenon similar to that seen in apocrine/eccrine glands adjacent to squamous cell carcinoma. In a limited biopsy specimen, these areas should not be mistaken for adenocarcinoma.