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Abstract Background Apocrine carcinoma associated with Paget’s disease is a rare malignancy that typically manifests in elderly individuals, predominantly affecting the geriatric population. It commonly arises in regions rich in apocrine glands and often exhibits an insidious onset, potentially requiring several years to be diagnosed. Case presentation An 80-year-old male was simultaneously diagnosed with scrotal apocrine carcinoma (showing Paget changes) and early-stage gastric cancer. Whole-genome exome sequencing confirmed these as independent malignancies with minimal genetic overlap, indicating that they were two primary tumors. The patient initially underwent successful surgery but experienced recurrence and metastasis. Treatment with capecitabine and paclitaxel showed promising responses, highlighting similarities between breast and apocrine carcinomas. Challenges were noted in the use of genetic testing and drug susceptibility assessments for treatment guidance. Notably, HER-2 expression in metastatic lesions, a trait of apocrine carcinoma, has remained unexplored due to negative HER-2 FISH results and a lack of available targeted therapies in China. Conclusion Elderly patients often exhibit a lesser degree of aggressiveness toward treatment following a diagnosis of malignant tumors. It is imperative to carefully consider how to strike a balance between effective treatment and maintaining a satisfactory quality of life for these patients. This case underscores the complexity of treating coexisting rare cancers in older adults and emphasizes the need for personalized treatments and continued innovation in cancer therapy. The insights gained offer significant value in understanding and managing such rare cancer cases.

Background: Apocrine carcinoma associated with Paget's disease is a rare malignancy that typically manifests in elderly individuals, predominantly affecting the geriatric population. It commonly arises in regions rich in apocrine glands and often exhibits an insidious onset, potentially requiring several years to be diagnosed. Case presentation: An 80-year-old male was simultaneously diagnosed with scrotal apocrine carcinoma (showing Paget changes) and early-stage gastric cancer. Whole-genome exome sequencing confirmed these as independent malignancies with minimal genetic overlap, indicating that they were two primary tumors. The patient initially underwent successful surgery but experienced recurrence and metastasis. Treatment with capecitabine and paclitaxel showed promising responses, highlighting similarities between breast and apocrine carcinomas. Challenges were noted in the use of genetic testing and drug susceptibility assessments for treatment guidance. Notably, HER-2 expression in metastatic lesions, a trait of apocrine carcinoma, has remained unexplored due to negative HER-2 FISH results and a lack of available targeted therapies in China. Conclusion: Elderly patients often exhibit a lesser degree of aggressiveness toward treatment following a diagnosis of malignant tumors. It is imperative to carefully consider how to strike a balance between effective treatment and maintaining a satisfactory quality of life for these patients. This case underscores the complexity of treating coexisting rare cancers in older adults and emphasizes the need for personalized treatments and continued innovation in cancer therapy. The insights gained offer significant value in understanding and managing such rare cancer cases. [ABSTRACT FROM AUTHOR]

Purpose of review: The purpose of this review is to provide an overview of ten unique breast cancer subtypes and their clinicopathologic features and treatment implications. Recent findings: Recent findings show that while many subtypes (mucinous, papillary, tubular, apocrine) have favorable biology, with better overall survival than invasive ductal carcinoma, some (metaplastic, adenoid cystic) are more aggressive portending worse prognosis for patients. Summary: The differences in histology represented in these breast cancer subtypes often impacts biology, behavior, and prognosis. Due to their rarity, additional research is needed to implement clear treatment protocols for each subtype. [ABSTRACT FROM AUTHOR]

Summary Apocrine metaplastic change is a frequent change in breast pathology. Invasive carcinoma with apocrine differentiation of the breast has unconventional histology, immunohistochemical (IHC), and molecular profile. It has an Estrogen receptor (ER)/Progesterone receptor (PR) negative and an Androgen receptor (AR) positive profile. About 1/3 of cases can show Her2neu amplification through IHC or Fluorescence in situ hybridization (FISH). Lymphovascular invasion (LVI) and lymph node metastasis (LNM) are frequently observed and they often have poor pathological response to chemotherapy. Histologically and molecularly defined apocrine subtypes of breast cancer, although have considerable overlap, yet are different and discrete entities. The decision on using chemotherapy and targeted regimens in these lesions is still controversial which calls for more insight. This could be achieved by acquiring a standardized diagnostic practice, further research, and discussion.

Apocrine carcinoma cases with sebaceous differentiation have not been reported and can be misdiagnosed as sebaceous carcinoma. We present two cases of apocrine carcinoma with marked sebocyte‐like cytological features. Tumors were observed in the left axilla of a 68‐year‐old man (Case 1) and the right axilla of a 72‐year‐old man (Case 2). Both patients presented with multiple lymph node metastases. Histopathology revealed densely distributed solid nests of tumor cells containing foamy cytoplasm and enlarged round nuclei with prominent nucleoli. The tumor cells diffusely expressed adipophilin, PRAME (cytoplasmic pattern), androgen receptor, BerEP4, and GCDFP15 but did not express p63 in both cases. PIK3CA E726K and H1047R mutations were detected in Cases 1 and 2, respectively. Tumor location in the axilla, the presence of eosinophilic granular cytoplasm, prominent nucleoli, and PIK3CA mutations, immunoreactivity for BerEP4 and GCDFP15, and lack of p63 immunoexpression findings matched apocrine carcinoma characteristics, but not sebaceous carcinoma. Thus, apocrine carcinoma can demonstrate intracytoplasmic lipid accumulation and rarely exhibit sebocyte‐like cytological features. Apocrine carcinoma should be distinguished from sebaceous carcinoma due to the former's higher metastatic potential and lack of association with Muir–Torre syndrome. [ABSTRACT FROM AUTHOR]

Apocrine carcinoma of the breast is a rare subtype of breast carcinoma, which only presents as 4% among patients with breast cancer. The percentage varies based on the diagnostic criteria used by each institution to classify apocrine carcinoma. Several confusing terms used in previous studies, including apocrine ductal carcinoma in situ (ADCIS), apocrine morphology in lobular carcinoma in situ (Apocrine LCIS), apocrine-like invasive carcinoma, pure apocrine carcinoma, molecular apocrine tumors (MATs), and triple-negative apocrine carcinoma of the breast (TNAC). The treatment, prognosis, and molecular profiles are also diverse. Pure apocrine carcinoma has stricter criteria for diagnosis, requiring more than 90% of cells showing apocrine morphology and classic IHC characteristics of ER-negative, PR-negative, and AR-positive in at least 10% of tumor cell nuclei. Research related to prognosis is diverse due to the difficulty of unifying the diagnostic criteria. Current evidence of treatment is geared toward the use of neoadjuvant chemotherapy and anti-androgen therapy when AR is present, accompanied by other treatments if biomarkers are present, such as HER2, PI3K, or CDK4/6. This article focuses on clearly summarizing different subtypes and management of apocrine carcinoma of the breast.

In the current study, we assessed the prevalence and molecular features of HER2-low phenotype in the apocrine carcinomas of the breast (ApoCa) and its relationship with tumor-infiltrating lymphocytes (TILs). A cohort of 64 well-characterized therapy-naïve ApoCa was used. The TIL distribution was assessed using the hematoxylin and eosin whole slide/scanned images following the international TILs working group recommendations. Next-generation sequencing (NGS) was performed in a subset of HER2-low ApoCa. All patients were women, with a mean age of 62 years. Forty-three carcinomas were pure apocrine carcinoma (PAC; ER−/AR+), and the remaining 21 were classified as apocrine-like carcinomas (ALCs; ER+/−, AR+/−). HER2/neu was positive (score 3+ by IHC and/or amplified by FISH) in 20/43 (47%) PAC and 4/21 (19%) ALC. The prevalence of HER2-low expression (scores 1+ or 2+ without HER2 amplification) in ApoCa was 39% without significant differences between PAC and ALC (P = 0.14); however, the HER2-low phenotype was more prevalent in triple-negative PAC than in ALC (P < 0.001). Levels of TILs were low (≤10%) in 74% of ApoCa (median 5%, range 0%–50%). TIL levels were significantly higher in ALC than in PAC (P = 0.02). HER2 status had no impact on TIL distribution (P = 0.45). The genomic profile of HER2-low ApoCa was similar to other subtypes of ApoCa. ApoCa has predominantly low TIL, particularly PAC. The prevalence of the HER2-low phenotype in ApoCa is high, which should have therapeutic and clinical implications given the recently approved therapies with antibody–drug conjugates (ADCs) for HER2-low breast cancers.

The differential diagnosis for an axillary mass in a patient with a previously treated malignancy is broad and definitive tissue diagnosis is required to guide treatment and surveillance strategies. We present the case of a 76-year-old African American male with a history of prostate cancer who presented with a left axillary mass two years after achieving remission from his prostate malignancy. Due to the diagnostic challenge, this excisional biopsy was reviewed at four different academic centers. Although no universal consensus among these institutions' pathologists, but in the context of clinical presentation and anatomic location, the overall clinical findings are consistent with apocrine sweat gland carcinoma. The mass was treated with complete local surgical excision, though regional lymph node metastasis occurred 2 years later. Multimodal treatment with surgery and radiation was done with removal of regional metastasis and no distant disease was identified. Primary apocrine carcinoma is a rare cutaneous neoplasm with less than 100 reported cases in the literature. A combination of clinical history and presentation, histomorphology, anatomical location, and immunohistochemistry is used to support the diagnosis and ultimately drive management.

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. [ABSTRACT FROM AUTHOR]

Haimanot Kasahun Alemu,1 Nazik Hammad,2 Mesfin Asefa Tola,3 Verna Vanderpuye4 1Department of Internal Medicine, Oncology Unit; Saint Paul’s Hospital Millennium Medical College, Addis Ababa, Ethiopia; 2Department of Medical Oncology, Queen’s University, Kingston, Ontario, Canada; 3Department of Pathology; Saint Paul’s Hospital Millennium Medical College, Addis Ababa, Ethiopia; 4National Centre for Radiotherapy, Oncology and Nuclear Medicine, Korle Bu Teaching Hospital, Accra, GhanaCorrespondence: Haimanot Kasahun Alemu, Clinical Oncologist, Saint Paul’s Hospital Millennium Medical College, Swaziland Street, PO Box: 13920/1271, Addis Ababa, Ethiopia, Tel +251 930594404, Email haimanotkasahun@gmail.comAbstract: We present a case report on a case of invasive apocrine carcinoma of breast during pregnancy at a tertiary referral hospital in Ethiopia. The patient’s case in this report signifies the challenging clinical situation that the patient, developing fetus and treating physicians have to go through and the need to improve maternal–fetal medicine and oncologic setup and treatment guidelines in Ethiopia. Our case also illustrates the huge disparity between the management of both breast cancer and its occurrence during pregnancy in low-income countries like Ethiopia and developed nations elsewhere. Our case report shows a rare histological finding. The patient has invasive apocrine carcinoma of the breast. To our knowledge, it is the first case to be reported in the country.Keywords: breast cancer, pregnancy, apocrine carcinoma, disparity, case report

Apocrine carcinoma is an extremely rare malignant cutaneous neoplasm that usually arises in areas with a high density of apocrine glands. Diagnosis can be challenging as tumours share histological and immunophenotypic characteristics with them. At first evaluation, the disease is often assumed to be benign. There have been approximately 100 reports of apocrine neoplasms in the literature. A 48-year-old male presented with a right axillary mass which increased in size over a period of 2 years. The patient was reported to have had ayurvedic therapy, but his swelling remained unchanged. Axillary lymph nodes were palpable. USG axilla suggested a well-defined fungating solid isoechoic lesion. USG neck did not reveal any abnormality. The mass was surgically excised as a whole by removing the overlying skin with margins and lymph node excision. The patient was diagnosed with primary apocrine carcinoma after surgical excision. The differentials include adenocarcinoma of breast and prostate and apocrine adenoma. There are no established standards for the care of this form of carcinoma due to its rarity and the absence of clinical studies. A literature evaluation and further reporting will aid in developing diagnostic standards and the most efficient treatment options.

Apocrine carcinoma is an extremely rare malignant cutaneous neoplasm that usually arises in areas with a high density of apocrine glands. Diagnosis can be challenging as tumours share histological and immunophenotypic characteristics with them. At first evaluation, the disease is often assumed to be benign. There have been approximately 100 reports of apocrine neoplasms in the literature. A 48-year-old male presented with a right axillary mass which increased in size over a period of 2 years. The patient was reported to have had ayurvedic therapy, but his swelling remained unchanged. Axillary lymph nodes were palpable. USG axilla suggested a well-defined fungating solid isoechoic lesion. USG neck did not reveal any abnormality. The mass was surgically excised as a whole by removing the overlying skin with margins and lymph node excision. The patient was diagnosed with primary apocrine carcinoma after surgical excision. The differentials include adenocarcinoma of breast and prostate and apocrine adenoma. There are no established standards for the care of this form of carcinoma due to its rarity and the absence of clinical studies. A literature evaluation and further reporting will aid in developing diagnostic standards and the most efficient treatment options.

Breast carcinoma is the most prevalent cancer among women globally. It has variable clinical courses depending on the stage and clinical-biological features. This case report describes a 56-year-old female with invasive breast cancer without estrogen or progesterone receptor expression, with apocrine differentiation, and with no germline variants in the BRCA1 and BRCA2 genes. Throughout the clinical course, the patient exhibited discordant results for HER2 in immunohistochemistry and in situ hybridization. During the second relapse, the disease displayed apocrine microscopic features. The tumor underwent analysis for the androgen receptor, GCDFP-15, RNA-seq, and whole-genome sequencing (WGS) to identify the breast cancer subtype and to characterize the cancer genome. Our bioinformatic analysis revealed 20,323 somatic SNV/Indels, including five mutations in cancer-related genes that are believed to be responsible for the tumor's development. Two of these mutations were found in the PIK3CA and TP53 genes. Furthermore, the tumor tissue exhibited large copy number alterations to the chromosomes, which could impact gene expression through complex mechanisms and contribute to the tumor phenotype. Clustering algorithms applied on RNA-sequencing data categorized this cancer as a HER2+ subtype. The second-line capecitabine chemotherapy treatment is ongoing, and the patient is responding well. Bioinformatic results support the current treatment decision and open the way to further treatments. [ABSTRACT FROM AUTHOR]

Apocrine carcinoma is an extremely rare malignant cutaneous neoplasm that usually arises in areas with a high density of apocrine glands. 1 Diagnosis can be challenging as tumours share histological and immunophenotypic characteristics with them. At first evaluation, the disease is often assumed to be benign. There have been approximately 100 reports of apocrine neoplasms in the literature. A 48-year-old male presented with a right axillary mass which increased in size over a period of 2 years. The patient was reported to have had ayurvedic therapy, but his swelling remained unchanged. Axillary lymph nodes were palpable. USG axilla suggested a well-defined fungating solid isoechoic lesion. USG neck did not reveal any abnormality. The mass was surgically excised as a whole by removing the overlying skin with margins and lymph node excision. The patient was diagnosed with primary apocrine carcinoma after surgical excision. The differentials include adenocarcinoma of breast and prostate and apocrine adenoma. There are no established standards for the care of this form of carcinoma due to its rarity and the absence of clinical studies. A literature evaluation and further reporting will aid in developing diagnostic standards and the most efficient treatment options.

Apocrine carcinoma is an extremely rare malignant cutaneous neoplasm that usually arises in areas with a high density of apocrine glands. 1 Diagnosis can be challenging as tumours share histological and immunophenotypic characteristics with them. At first evaluation, the disease is often assumed to be benign. There have been approximately 100 reports of apocrine neoplasms in the literature. A 48-year-old male presented with a right axillary mass which increased in size over a period of 2 years. The patient was reported to have had ayurvedic therapy, but his swelling remained unchanged. Axillary lymph nodes were palpable. USG axilla suggested a well-defined fungating solid isoechoic lesion. USG neck did not reveal any abnormality. The mass was surgically excised as a whole by removing the overlying skin with margins and lymph node excision. The patient was diagnosed with primary apocrine carcinoma after surgical excision. The differentials include adenocarcinoma of breast and prostate and apocrine adenoma. There are no established standards for the care of this form of carcinoma due to its rarity and the absence of clinical studies. A literature evaluation and further reporting will aid in developing diagnostic standards and the most efficient treatment options.

Breast carcinoma is the most prevalent cancer among women globally. It has variable clinical courses depending on the stage and clinical-biological features. This case report describes a 56-year-old female with invasive breast cancer without estrogen or progesterone receptor expression, with apocrine differentiation, and with no germline variants in the BRCA1 and BRCA2 genes. Throughout the clinical course, the patient exhibited discordant results for HER2 in immunohistochemistry and in situ hybridization. During the second relapse, the disease displayed apocrine microscopic features. The tumor underwent analysis for the androgen receptor, GCDFP-15, RNA-seq, and whole-genome sequencing (WGS) to identify the breast cancer subtype and to characterize the cancer genome. Our bioinformatic analysis revealed 20,323 somatic SNV/Indels, including five mutations in cancer-related genes that are believed to be responsible for the tumor’s development. Two of these mutations were found in the PIK3CA and TP53 genes. Furthermore, the tumor tissue exhibited large copy number alterations to the chromosomes, which could impact gene expression through complex mechanisms and contribute to the tumor phenotype. Clustering algorithms applied on RNA-sequencing data categorized this cancer as a HER2+ subtype. The second-line capecitabine chemotherapy treatment is ongoing, and the patient is responding well. Bioinformatic results support the current treatment decision and open the way to further treatments.

Apocrine carcinoma of the breast is a rare subtype of breast carcinoma, which only presents as 4% among patients with breast cancer. The percentage varies based on the diagnostic criteria used by each institution to classify apocrine carcinoma. Several confusing terms used in previous studies, including apocrine ductal carcinoma in situ (ADCIS), apocrine morphology in lobular carcinoma in situ (Apocrine LCIS), apocrine-like invasive carcinoma, pure apocrine carcinoma, molecular apocrine tumors (MATs), and triple-negative apocrine carcinoma of the breast (TNAC). The treatment, prognosis, and molecular profiles are also diverse. Pure apocrine carcinoma has stricter criteria for diagnosis, requiring more than 90% of cells showing apocrine morphology and classic IHC characteristics of ER-negative, PR-negative, and AR-positive in at least 10% of tumor cell nuclei. Research related to prognosis is diverse due to the difficulty of unifying the diagnostic criteria. Current evidence of treatment is geared toward the use of neoadjuvant chemotherapy and anti-androgen therapy when AR is present, accompanied by other treatments if biomarkers are present, such as HER2, PI3K, or CDK4/6. This article focuses on clearly summarizing different subtypes and management of apocrine carcinoma of the breast. [ABSTRACT FROM AUTHOR]

Encapsulated papillary carcinoma is a special type of breast cancer defined in the fifth edition of the World Health Organization breast tumor classification guidelines. Apocrine encapsulated papillary carcinoma is extremely rare, and only 10 cases have been described previously. We encountered a case of apocrine encapsulated papillary carcinoma with frank invasion. The patient was a 77-year-old woman with a painless mass in her right breast. Core needle biopsy revealed that the tumor cells had voluminous eosinophilic cytoplasm and enlarged nuclei with prominent nucleoli. We diagnosed this lesion as carcinoma with apocrine differentiation and suggested the possibility of an encapsulated papillary carcinoma. The patient underwent a right-sided mastectomy. Gross examination of the resected specimen revealed a multilobulated tumor. Microscopically, the tumor cells, which had voluminous eosinophilic cytoplasm and enlarged nuclei with prominent nucleoli, proliferated in papillary fashion with fibrous stalks in the cystic space. Myoepithelial cells were not observed around the cystic space. Frank invasion was also observed around the encapsulated papillary carcinoma. Immunohistochemistry analysis revealed that the tumor cells were negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 and positive for androgen receptor and gross cys-tic disease fluid protein 15. Based on these findings, we diagnosed this lesion as an apocrine encapsulated papillary carcinoma with frank invasion. [ABSTRACT FROM AUTHOR]

Abstract Background Apocrine carcinoma is a rare tumor that constitutes < 4% of all breast malignancies, characterized by the proliferation of large atypical cells with strictly defined borders, abundant eosinophilic cytoplasm, large nuclei, and prominent nucleoli in more than 90% of tumor cells. Triple-negative apocrine carcinoma is a rare molecular subtype that constitutes less than 1% of triple-negative breast cancers and is characterized by negative expression of estrogen receptor, progesterone receptor, and human epidermal growth factor receptor, with positive expression of androgen receptor. Case presentation We report a case of a 45-year-old Syrian female who presented to our hospital due to a painless palpable mass in her left breast. Following physical and radiological examinations, an excisional biopsy was performed. Microscopic examination of the specimen followed by immunohistochemical staining revealed the diagnosis of a triple-negative apocrine carcinoma. Conclusion Triple-negative apocrine carcinoma is an extremely rare neoplasm that must be considered in the differential diagnoses of breast lesions through detailed clinical, histological, and immunohistochemical correlations. In our manuscript, we aimed to present the first case report of a Syrian female who was diagnosed with a triple-negative apocrine carcinoma, aiming to highlight the importance of detailed clinical, histological and immunohistochemical correlations with a detailed review of diagnostic criteria, molecular characteristics, and treatment recommendations.

Purpose: Apocrine carcinoma of the breast (APO) expresses HER2 in 30–50% of cases. This study explored the clinicopathological features and outcome of HER2+/APO and matched HER2+/NST cohort. Methods: We used the SEER database to explore the cohorts. Univariate and multivariate analyses were used to assess the survival. Based on ER and PR [steroid receptors/SR/] and HER2 status, we divided the cohorts to match the intrinsic molecular subtypes for comparisons. Results: We retrieved 259 cases of HER2+/APO. Most HER2+/APO were SR negative (65%). HER2+/APO were more prevalent in the 80+ age group (24.7% vs. 15.7%, p < 0.001). HER2+/SR−/APO had a significantly lower histological grade than the HER2+/SR−/NST (p < 0.001). Breast cancer-related deaths were more prevalent in HER2+/NST (7.8% vs. 3.9%, p = 0.019). This was particularly evident between SR− subgroups (10.4% in HER2+/SR−/NST vs. 4.2% in HER2+/SR−/APO, p = 0.008) and was reaffirmed in breast cancer-specific survival in univariate analysis (p = 0.03). Other than race and SR status, HER2+/APO subgroups did not differ in clinicopathological parameters. Conclusions: Our study confirms the rarity of the APO and reveals that SR status in APO does not affect these patients' prognosis. HER2+/APO tumors tend to have a less aggressive phenotype and a more favorable outcome despite a markedly lower ER/PR positivity. [ABSTRACT FROM AUTHOR]

Background: Triple negative breast cancer encompasses several biological entities with different outcomes and is a priority to identify which patients require more treatment to reduce the risk of recurrence and which patients need less treatment. Patients and methods: Among the 210 women with first primary invasive apocrine non metastatic breast cancer operated on between January 1998 and December 2016 at the European Institute Oncology, Milan, we identified 24 patients with a pT1-pT2, node-negative, triple negative subtype and Ki-67 ≤ 20% who did not receive adjuvant chemotherapy (CT). We compared the outcome of this cohort with a similar group of 24 patients with ductal tumors who received adjuvant chemotherapy, matched by pathological stage and biological features and also with a similar group of 12 patients with apocrine tumors who received adjuvant chemotherapy. Results: The median age was 64 and 61 years in the apocrine (w/o CT) and ductal group, respectively. The median value of Ki-67 expression was 12% in the apocrine group (w/o CT) and 16% in the ductal group (p

A breast mass in women often presents a diagnostic challenge due to the diversity in the diagnosis. We herein report a rare variant of breast carcinoma, an invasive apocrine carcinoma (AC), in an elderly woman where the breast mass clinically mimicked phyllodes tumour. Immunohistochemistry (IHC) showed the tumour as triple-negative and also negative for androgen receptor (AR). Gross cystic disease fluid protein (GCDFP-15) was strongly and diffusely positive. It is an exceptional finding. It implies its significance as a diagnostic marker of AC of the breast. The accurate diagnostic criteria of AC are still lacking. Patients with breast lumps offer unique challenges and enormous responsibility to primary and family care physicians.

Primary cutaneous apocrine carcinomas are rare cutaneous adnexal neoplasm with less than 100 cases reported in the literature. The tumor arises in areas rich in apocrine glands particularly axilla, modified apocrine glands such as ceromucinous and Moll's glands. Most of these carcinomas are indolent and slowly growing but few rapidly progressive and extremely aggressive? cases have also been reported. Wide local excision with clear margins and sentinel lymph node biopsy is standard treatment of choice.This case was of much interest from the diagnostic point of view both microscopically as well as immunohistochemically. We report a case of 46-year-old man who presented to our hospital with an ulcerated painless nodular mass in his right axilla.With combined use of microscpic features, special stains and immunohistochemical examination the case was correctly diagnosed as primary cutaneous apocrine carcinoma.

Aims: The aim of this study was to determine the clinicopathological and genetic characteristics of axillary signet‐ring cell/histiocytoid carcinoma (SRCHC) and the relationship between axillary SRCHC, eyelid SRCHC, and conventional apocrine carcinoma (AC). Methods and results: Eleven cases of axillary SRCHC, four cases of eyelid SRCHC, eight cases of axillary AC and five cases of invasive lobular carcinoma (ILC) were retrieved. Additionally, 14 axillary and 43 eyelid SRCHC cases from the literature were reviewed. Male predominance was prominent for axillary SRCHC (24:1) and eyelid SRCHC (42:5). Axillary SRCHC formed a circumscribed plaque or nodule, unlike eyelid SRCHC. Lymph node metastasis was predominantly seen in axillary SRCHC cases (72%, 18/25), but not in eyelid SRCHC cases (19%, 9/47). Axillary SRCHC and eyelid SRCHC were histopathologically similar and showed rare tubular formations. Immunoexpression of cytokeratin 7, cytokeratin 19, mucin 1, mucin 5AC, BerEP4 and androgen receptor was seen in all tested cases of the four diseases. Oestrogen and progesterone receptors were negative in both types of SRCHC and AC, but were strongly positive in ILCs. Complete loss of E‐cadherin expression was seen in approximately one‐quarter of both types of SRCHC and in all ILCs. PIK3CA mutations were detected in all three sequenced cases (two axillary SRCHCs and one eyelid SRCHC). Conclusion: The histopathological, immunohistochemical and genetic findings suggest that both types of SRCHC are phenotypic variants of AC, although there are differences in sex, macroscopic findings and the frequency of lymph node metastasis among the three. In contrast, ILC differs from the other three tumour types. [ABSTRACT FROM AUTHOR]

Background: Apocrine carcinoma is a rare tumor that constitutes < 4% of all breast malignancies, characterized by the proliferation of large atypical cells with strictly defined borders, abundant eosinophilic cytoplasm, large nuclei, and prominent nucleoli in more than 90% of tumor cells. Triple-negative apocrine carcinoma is a rare molecular subtype that constitutes less than 1% of triple-negative breast cancers and is characterized by negative expression of estrogen receptor, progesterone receptor, and human epidermal growth factor receptor, with positive expression of androgen receptor.Case Presentation: We report a case of a 45-year-old Syrian female who presented to our hospital due to a painless palpable mass in her left breast. Following physical and radiological examinations, an excisional biopsy was performed. Microscopic examination of the specimen followed by immunohistochemical staining revealed the diagnosis of a triple-negative apocrine carcinoma.Conclusion: Triple-negative apocrine carcinoma is an extremely rare neoplasm that must be considered in the differential diagnoses of breast lesions through detailed clinical, histological, and immunohistochemical correlations. In our manuscript, we aimed to present the first case report of a Syrian female who was diagnosed with a triple-negative apocrine carcinoma, aiming to highlight the importance of detailed clinical, histological and immunohistochemical correlations with a detailed review of diagnostic criteria, molecular characteristics, and treatment recommendations. [ABSTRACT FROM AUTHOR]

Cutaneous apocrine carcinomas share common features with their counterparts in the breast; hence, metastatic mammary carcinoma must be excluded before such lesions can be designated primary cutaneous neoplasms. Primary tumors from either source rarely exhibit neuroendocrine differentiation. We report a case of a 72‐year‐old female with a painless 1.2‐cm scalp nodule. An incisional biopsy revealed dermal involvement by an invasive apocrine carcinoma juxtaposed to a benign apocrine cystic lesion. Immunohistochemically, the carcinoma expressed neuroendocrine proteins including synaptophysin, chromogranin, and CD56. A primary cutaneous apocrine carcinoma with neuroendocrine differentiation was favored, but additional investigations to exclude breast origin were recommended. These revealed a 1.1‐cm nodule in the right breast, which proved to be an invasive ductal carcinoma, morphologically and immunophenotypically similar to the scalp lesion. This confounded the case, yet factors militating against metastatic breast carcinoma to skin included (a) the small size of the mammary tumor, (b) absence of other metastatic disease, and (c) juxtaposition of the scalp carcinoma to a putative benign precursor. Molecular studies were undertaken to resolve the diagnostic quandary. Single nucleotide polymorphism microarray analysis revealed distinct patterns of chromosomal copy number alterations in the two tumors, supporting the concept of synchronous and unusual primary neoplasms. [ABSTRACT FROM AUTHOR]

Abstract Background Triple‐negative apocrine carcinoma (TNAC) of the breast is a very rare type of breast cancer. Furthermore, the clinicopathological features, prognosis, and potential impact of treatment strategies in TNAC remain unclear. Methods Data from the Surveillance, Epidemiology, and End Results (SEER) program were used to identify breast cancer patients diagnosed between 2010 and 2016 with TNAC and triple‐negative breast cancer (TNBC, IDC [invasive ductal carcinoma], NOS [not otherwise specified]). Chi‐squared tests were used to examine the categorical variables between the two groups. Overall survival (OS) of TNAC and TNBC was assessed by Kaplan‐Meier analyses and Cox regression. Breast cancer‐specific survival (BCSS) was evaluated by Nelson‐Aalen analyses and competing risk regression. Results We identified 31 362 patients from the SEER database, including 366 patients with TNAC and 30 996 patients with TNBC. TNAC was correlated with older age, lower T stage and lower tumor grade. Patients with TNAC had better OS compared with TNBC patients; the 5‐year OS rates were 82.2% vs 73.5% (P

Objective: Extramammary Paget's disease (EMPD) with invasive carcinoma and distant metastasis is extremely rare. In vulva EMPD associated apocrine carcinoma with signet ring cell differentiation has not been described in the literature so far. Its slow evolution, varied clinical presentation and histological appearances, lead to difficulty in diagnosis of this disease. Case report: We hereby report a case of primary EMPD with invasive carcinoma and distant metastasis in a 59-year-old female who presented with erythematous indurated plaque over vulva. Histopathology revealed Paget cell infiltration throughout the epidermis with invasive carcinoma in dermis and liver metastasis on CECT. The immunohistochemical expressions of CK7, CK20, GCDFP-15, CEA, p40, CDX 2, Her-2/ neu, AR, ER, were examined to explicate the cellular differentiation of this carcinoma. According to the histological assessment, this case was diagnosed as primary EMPD with apocrine adenocarcinoma, signet ring cell differentiation, vulva. Conclusion: Owing to poor prognosis, a high index of clinical suspicion along with histological and immunohistochemical assessment is of utmost importance in arriving at final diagnosis.

Primary cutaneous apocrine carcinoma (PCAC) is an extremely rare malignancy. Distinguishing apocrine carcinoma from breast carcinoma metastasis is difficult. Only a few cases reported as PCAC of the scalp and primarily treated by wide local excision. The usual presentation is a skin lesion that rapidly progresses over the duration of a few weeks to few months. We reported a 56-year-old man with a right scalp ulcerative lesion diagnosed as a CAC. The patient underwent wide local excision. 1.5 years later, the patient developed neck lymph node metastasis that treated with neck dissection and adjuvant radiotherapy. No chemotherapy was given due to limited literature, suggesting substantial benefits of adjuvant chemotherapy for such cases. Review literature was performed to assess the clinical presentation, treatment, and prognosis of such malignancies. PCAC of the scalp is a challenging malignancy in the diagnosis and management.

Apocrine Carcinoma (APC) is a rare and distinct entity among all the histological variants of invasive ductal carcinoma(IDC) of breast. It is common to encounter focal apocrine differentiation, but only 4% of IDCs show extensive apocrine differentiation. In the 2012 WHO classification of tumors of breast, it is placed under specialized subtypes of IDC and named “carcinomas with apocrine differentiation.” Apocrine differentiation is seen with IDC-no specific type (IDC-NST) as well as in special type carcinomas such as tubular, lobular, micropapillary, and medullary and that's why it does not represent as a separate entity. Although clinical, radiological, and gross appearance is indistinguishable from IDC-NST (the most common form), APC has a typical histomorphological picture with distinct molecular apocrine signature and immunohistochemistry. It is characterized by increased androgen expression overlapping with HER2/neu expression and estrogen receptor (ER) as well as progesterone receptor (PR) negativity. It is thought to have worse prognosis; hence, diagnosing this entity at the earliest is very important for implementation of new treatment modalities. Here, we report two such cases of carcinomas with apocrine features – 47-year-old and 52-year-old patients showing features of apocrine carcinoma and positivity for androgen receptor with negativity for ER and PR.

Primary cutaneous mucinous carcinoma (PCMC) is an uncommon tumor of the sweat gland origin. The occurrence of PCMC is mostly in middle-aged and older patients, with a slight male predominance. Most cases of PCMC arise on the head, with a preference for eyelids. The histogenesis of PCMC, whether eccrine or apocrine, remains controversial. We report a rare case of PCMC with secondary extramammary Paget’s disease in the groin of a 75-year-old man, which favored an apocrine origin. Furthermore, based on a review of the literature, we provide several histologic clues that can be used to differentiate PCMC from metastatic mucinous carcinoma.

Objective: Extramammary Paget's disease (EMPD) with invasive carcinoma and distant metastasis is extremely rare. In vulva EMPD associated apocrine carcinoma with signet ring cell differentiation has not been described in the literature so far. Its slow evolution, varied clinical presentation and histological appearances, lead to difficulty in diagnosis of this disease. Case report: We hereby report a case of primary EMPD with invasive carcinoma and distant metastasis in a 59-year-old female who presented with erythematous indurated plaque over vulva. Histopathology revealed Paget cell infiltration throughout the epidermis with invasive carcinoma in dermis and liver metastasis on CECT. The immunohistochemical expressions of CK7, CK20, GCDFP-15, CEA, p40, CDX 2, Her-2/ neu, AR, ER, were examined to explicate the cellular differentiation of this carcinoma. According to the histological assessment, this case was diagnosed as primary EMPD with apocrine adenocarcinoma, signet ring cell differentiation, vulva. Conclusion: Owing to poor prognosis, a high index of clinical suspicion along with histological and immunohistochemical assessment is of utmost importance in arriving at final diagnosis. [ABSTRACT FROM AUTHOR]

• Primary apocrine sweat gland carcinoma (PASGC) is a rare subtype of sweat gland carcinoma, mostly occurring in the apocrine-dense regions. • The role of sentinel lymph node biopsy (SLNB) for axillary PASGC treatment remains controversial because of its rarity. • In axillary cases, to identify SLNs could be difficult because the lymphatic flow of the axillary skin is complicated, unlike the arm and areola. • Although SLNB in sweat gland carcinoma has long been known and used, this is the first axillary PASGC case that was performed SLNB. Primary apocrine sweat gland carcinoma (PASGC) is a rare subtype of sweat gland carcinoma, mostly occurring in the apocrine-dense regions such as the axilla, areola, and scalp. An 83-year-old woman developed a red elevating mass on her left axilla, without palpable axillary lymph nodes. Excision biopsy revealed that the tumor was PASGC. Additional wide resection and sentinel lymph node biopsy (SLNB) were performed without any adjuvant therapy. One year after the surgical procedure, the patient did not show any evidence of recurrence and axillary surgical complications. As PASGC arising in the axilla can progress aggressively, differential diagnosis is essential. Previous reports have shown the usefulness of SLNB, but the axillary management for patients with clinically node negative PASGC has not been established because of its rarity. Especially in axillary cases, to identify the true sentinel lymph nodes is sometimes considered inaccurate because the lymphatic flow is complicated in the axilla. Although SLNB in sweat gland carcinoma has believed safety and been performed, this is the first axillary PASGC case that was performed SLNB. Careful follow-up is needed. [ABSTRACT FROM AUTHOR]

Triple negative breast cancer encompasses several biological entities with different outcomes and is a priority to identify which patients require more treatment to reduce the risk of recurrence and which patients need less treatment. Among the 210 women with first primary invasive apocrine non metastatic breast cancer operated on between January 1998 and December 2016 at the European Institute Oncology, Milan, we identified 24 patients with a pT1-pT2, node-negative, triple negative subtype and Ki-67 ≤ 20% who did not receive adjuvant chemotherapy (CT). We compared the outcome of this cohort with a similar group of 24 patients with ductal tumors who received adjuvant chemotherapy, matched by pathological stage and biological features and also with a similar group of 12 patients with apocrine tumors who received adjuvant chemotherapy. The median age was 64 and 61 years in the apocrine (w/o CT) and ductal group, respectively. The median value of Ki-67 expression was 12% in the apocrine group (w/o CT) and 16% in the ductal group (p < 0.001). After a median follow-up of 7.5 years, no patients in the apocrine group (w/o CT) experienced a breast cancer related event compared with 4 events in the ductal carcinoma group (Gray test p-value = 0.11). The outcome of selected apocrine triple negative breast cancer patients who did not received adjuvant chemotherapy is excellent and supports a treatment de-escalation. Multicenter projects focusing on the possibility of avoiding adjuvant chemotherapy in selected subtypes of triple negative breast cancers with favorable outcome are warranted. • Triple negative breast cancer is a eterogeneous disease. • The outcome of selected apocrine triple negative breast cancer patients is excellent despite the omission of chemotherapy. • A treatment de-escalation should be discussed. • Multicenter projects focusing on selected subtypes of triple negative breast cancers are warranted. [ABSTRACT FROM AUTHOR]

Invasive apocrine carcinoma of the breast is rare. The criteria for the diagnosis are uncertain with the variable reported incidence in the literature. The use of androgen receptor studies by immunohistochemistry and its potential response to androgen analogs necessitates the subtyping of this tumor. We report a case of giant invasive apocrine carcinoma of the breast involving the entire breast parenchyma without skin and chest wall infiltration. The clinical, pathological, immunohistochemical, and ultrastructural findings are discussed with emphasis on morphological and immunohistochemical features that aid in the diagnosis of this rare tumor.

A breast mass in women often presents a diagnostic challenge due to the diversity in the diagnosis. We herein report a rare variant of breast carcinoma, an invasive apocrine carcinoma (AC), in an elderly woman where the breast mass clinically mimicked phyllodes tumour. Immunohistochemistry (IHC) showed the tumour as triple-negative and also negative for androgen receptor (AR). Gross cystic disease fluid protein (GCDFP-15) was strongly and diffusely positive. It is an exceptional finding. It implies its significance as a diagnostic marker of AC of the breast. The accurate diagnostic criteria of AC are still lacking. Patients with breast lumps offer unique challenges and enormous responsibility to primary and family care physicians. [ABSTRACT FROM AUTHOR]

Background: Triple‐negative apocrine carcinoma (TNAC) of the breast is a very rare type of breast cancer. Furthermore, the clinicopathological features, prognosis, and potential impact of treatment strategies in TNAC remain unclear. Methods: Data from the Surveillance, Epidemiology, and End Results (SEER) program were used to identify breast cancer patients diagnosed between 2010 and 2016 with TNAC and triple‐negative breast cancer (TNBC, IDC [invasive ductal carcinoma], NOS [not otherwise specified]). Chi‐squared tests were used to examine the categorical variables between the two groups. Overall survival (OS) of TNAC and TNBC was assessed by Kaplan‐Meier analyses and Cox regression. Breast cancer‐specific survival (BCSS) was evaluated by Nelson‐Aalen analyses and competing risk regression. Results: We identified 31 362 patients from the SEER database, including 366 patients with TNAC and 30 996 patients with TNBC. TNAC was correlated with older age, lower T stage and lower tumor grade. Patients with TNAC had better OS compared with TNBC patients; the 5‐year OS rates were 82.2% vs 73.5% (P <.001). The breast cancer‐related death rate was significantly lower in patients with TNAC than in patients with TNBC, with a 5‐year cumulative incidence of 9.1% vs 22.9% (P <.001). Chemotherapy was significantly associated with improved OS in TNAC patients, but radiotherapy was not associated with OS in TNAC patients. In the multivariable Cox regression, TNAC was still associated with improved OS (HR [hazard ratio], 0.61; 95% CI [confidence interval] 0.45‐0.83; P =.002). In the multivariable competing risk regression, the significantly higher BCSS in patients with TNAC compared patients with TNBC remained (subdistribution HR [SHR], 0.42; 95% CI, 0.27‐0.64; P <.001). Conclusion: Patients with TNAC had a better prognosis than patients with TNBC, and chemotherapy was associated with survival advantages in TNAC patients. [ABSTRACT FROM AUTHOR]

Primary cutaneous apocrine carcinoma (PCAC) is an extremely rare malignancy. Distinguishing apocrine carcinoma from breast carcinoma metastasis is difficult. Only a few cases reported as PCAC of the scalp and primarily treated by wide local excision. The usual presentation is a skin lesion that rapidly progresses over the duration of a few weeks to few months. We reported a 56-year-old man with a right scalp ulcerative lesion diagnosed as a CAC. The patient underwent wide local excision. 1.5 years later, the patient developed neck lymph node metastasis that treated with neck dissection and adjuvant radiotherapy. No chemotherapy was given due to limited literature, suggesting substantial benefits of adjuvant chemotherapy for such cases. Review literature was performed to assess the clinical presentation, treatment, and prognosis of such malignancies. PCAC of the scalp is a challenging malignancy in the diagnosis and management. [ABSTRACT FROM AUTHOR]

Pleomorphic lobular carcinoma (PLC) of the breast is a variant of lobular carcinoma, characterized by loss of E‐cadherin expression and high‐grade morphologies. Whether the pathogenesis of PLC is in the ductal or the lobular lineage has been discussed. In this report, a case of PLC combined with apocrine carcinoma is presented. Histologically, the tumor showed two distinct carcinoma components: one was a typical apocrine carcinoma, and the other was a pleomorphic invasive lobular carcinoma. The former showed complete membranous expression of E‐cadherin, whereas the latter aberrantly expressed it not on the cell membrane, but in the cytoplasm. Both components were triple‐negative and strongly positive for GCDFP‐15, suggesting apocrine differentiation. The intraductal component showed only a feature of apocrine ductal carcinoma in situ. This case suggests that apocrine carcinoma could be an origin of PLC. [ABSTRACT FROM AUTHOR]