A review of cutaneous apocrine carcinoma: epidemiology, diagnosis, prognosis, and treatment options.

Cutaneous apocrine carcinoma is a rare skin cancer arising from apocrine sweat glands. Disease-specific treatments are required for cutaneous adnexal carcinomas due to their heterogeneous treatment responsiveness. This review reports on the epidemiology, diagnosis, pathological features, surgical management, and use of systemic therapies for cutaneous apocrine carcinoma. Diagnosing cutaneous apocrine carcinoma requires presenting with distinctive pathological features and excluding metastatic adenocarcinomas, particularly breast cancer. Clinical findings are essential to exclude metastatic adenocarcinomas, and immunohistochemistry can be used as an adjunctive tool to rule out other diseases. Wide local excision is the standard treatment for resectable cutaneous apocrine carcinomas. Prophylactic lymphadenectomy should be considered as a treatment option given the high incidence of lymph node metastasis. Generally, cutaneous apocrine carcinomas are resistant to chemotherapy and radiation therapy; however, adjuvant radiotherapy is recommended for high-risk patients. Radiation or systemic therapy is administered to patients with distant metastases or recurrence. The systemic therapeutic options include cytotoxic chemotherapy, hormonal therapy, targeted therapy, and immune checkpoint inhibitors. Given the lack of data on clinical prognosis and standardized treatments, further studies are needed to improve our understanding of cutaneous apocrine carcinomas.

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.

Primary orbital apocrine adenocarcinoma: case report and literature review.

Primary apocrine adenocarcinoma (PAA) originating from the orbit is a rare malignant neoplasm. We present the case of a 61-year-old-male with PAA of the orbit. The patient underwent a right orbital exenteration, neck dissection, and adjuvant radiotherapy. Orbital exenteration is commonly performed as the primary intervention for PAA of the orbit. The role of adjuvant radiotherapy to prevent local recurrence is unclear and may be determined on a case-by-case basis.

Prognostic factors in cutaneous apocrine carcinoma: a single-institution retrospective study of 32 patients.

BACKGROUND: Cutaneous apocrine carcinoma (CAC) is a rare adnexal carcinoma. Limited data exists on the demographics and overall survival (OS) of patients with CAC; thus, there is no consensus on surgical management. This study aimed to examine demographic and survival data of patients with CAC to determine optimal surgical management. METHODS: A single-center retrospective cohort study was conducted at the National Cancer Center Hospital in Tokyo between 2005 and 2022. Patients with a histologically-confirmed CAC diagnosis were identified and data on patient demographics, OS, and lymph node (LN) status were gathered. RESULTS: Thirty-two patients were included (median age, 65.5 years; male-female ratio, 15:1). The most common involvement site was the axilla (87.5%). Of the nine patients in the clinical local stage, pathological LN metastases were found in five patients. Either pathological LN or distant metastases were present in 75% of the patients at initial diagnosis. The most common initial surgical treatments included wide local excision and complete LN dissection. Across cohorts, the median OS was 39 months. Patients with ≥ 4 LN metastases had reduced recurrence-free survival and OS compared to those with ≤ 3 LN metastases (p = 0.042, p = 0.041, respectively). The OS was not remarkably different between patients who did and did not receive postoperative radiation therapy. CONCLUSIONS: Since CAC has a high rate of LN metastasis-and the number of LN metastases is a significant prognostic factor-LN evaluation should be considered for patients with CAC as initial treatment. Nonetheless, ≥ 4 LN metastases can be a poor prognostic factor for CAC.

Primary apocrine adenocarcinoma of the lacrimal sac: an extremely rare variant of lacrimal sac neoplasm.

A 52-year-old male presented with right eye prominence of 1-year duration. Right eye examination revealed proptosis and limitation of extraocular movements. Magnetic resonance imaging (MRI) of the orbit showed soft tissue mass in the inferomedial orbit with medial rectus involvement and involving lacrimal sac. The patient underwent an incisional biopsy. Histopathology confirmed a diagnosis of primary orbital adenocarcinoma. Positron emission tomography computed tomography (PET CT) did not show the involvement of any other organ. Right orbital exenteration was done for disease control. On histopathology the mass was found to be arising from the lacrimal sac and was reported as apocrine adenocarcinoma. The diagnosis was further confirmed by immunohistochemistry with the tumor staining positive for gross cystic disease fluid protein 15 (GCDFP 15). The patient received adjuvant radiotherapy and is alive without any recurrence at 1 year of follow up. Apocrine adenocarcinomas are rare malignant skin adnexal tumors. Involvement of lacrimal sac is extremely rare with only one case reported in the literature till date. Immunohistochemistry utilizing GCDFP 15 stain can confirm the diagnosis.

A case report of a primary apocrine adenocarcinoma of the eyelid with literature review.

INTRODUCTION: Primary Apocrine adenocarcinomas (PAA) are very infrequent tumors that are often confused initially with benign lesions. Little is known about this disease and there is still much to be clarified. We present a case of PAA on the eyelid successfully treated with surgery alone and a literature review regarding what is currently described about this disease. METHODS: Noncomparative, retrospective case report of a patient with PAA on the eyelid succesfully treated with surgery alone and a literautre review. RESULTS: A 91-year-old man with a 2 months lesion on the upper left eyelid was treated with surgery alone with oncological margins of 5mm. The Hystopathology diagnosis was a PAA of the eyelid and free margins were obtained. After 12 months of follow-up, the patient does not show any signs of local recurrence or distant metastasis. A review of the literature suggests these tumors are located more frequently in the axilla (50%) and secondly in the head and neck (35%), with similar distribution in the upper (41%) and lower eyelid (45%). The most commonly used treatment is surgical excision, but radiotherapy and chemotherapy have also been used with variable results. CONCLUSIONS: PAA is a very rare and aggressive tumor. Because it is so infrequent, treatments are based on the sporadic cases encountered in the literature. As more cases are reported, more can be elucidated about the characteristics of this tumor, its behavior and best treatment choice and this may allow progress in the understanding and management of this disease.

Primary cutaneous apocrine carcinoma: A challenging case report.

INTRODUCTION: Primary cutaneous apocrine carcinomas of the axilla represents an extremely rare entity, with <200 cases reported in the literature. It can be challenging, even almost impossible, to distinguish histologically from metastases of breast origin. We herein present the first case of an axillary cutaneous apocrine adenocarcinoma followed and treated in our institute. CASE PRESENTATION: A 58-year-old man with a history of myopathy, presented for a right axillary swelling. Physical examination revealed the presence of a 10 cm right axillary mass, no palpable adenopathy, and bilateral gynecomastia. A biopsy of the mass was performed, showing a pattern consistent with a secondary localization of mammary neoplasia. Breast and distant radiological examinations were negative. The tumor markers' levels were not raised. Therefore, the patient underwent surgery with a large excision, a right axillary lymph node dissection, covered with a pedicled pectoralis major flap. Histological and immunohistochemical examinations showed a high expression of CK7 with a negative expression of TTF1, RH, PSA, and CK20. The diagnosis of an apocrine adenocarcinoma from cutaneous origin was confirmed. CLINICAL DISCUSSION: Primary cutaneous apocrine carcinomas are a group of uncommon malignant adnexal tumors, whose diagnosis is almost impossible to confirm preoperatively. Wide, local excision with clear margins, with or without lymph node dissection is the standard treatment. CONCLUSION: This case illustrates the importance of clinico-pathological correlation of skin cancers, especially apocrine ones. Clinical particularity and careful histological analysis are used to guide the diagnostic approach.

Vulval apocrine adenocarcinoma with tumour-associated eosinophilia in a cat.

A 10-year-old, spayed female, Domestic Shorthaired cat was referred for surgical removal of a mass on the left vulval fold. An impression smear revealed mixed cell inflammation, with eosinophils predominating focally, and a concurrent bacterial infection, suggesting a primarily inflammatory lesion. However, cytology of a fine-needle aspirate of the mass revealed a neoplastic epithelial cell population, confirmed on histopathology as an apocrine vulval adenocarcinoma with lymphatic invasion and marked tumour-associated tissue eosinophilia. One month after surgical excision of the mass, the cat developed inguinal metastatic lymphadenopathy and chemotherapy was initiated. The patient ultimately developed marked peripheral lymphadenomegaly and was euthanized due to concerns for overall quality of life and comfort. This case highlights that neoplasia should be a consistent differential diagnosis for eosinophilic infiltrates/inflammation. The distinct appearance of the two cytological samples in this case stresses the need for sampling of different sites of a lesion and the importance of not relying on superficial impression smears for clinical management and prognosis.

Apocrine adenocarcinoma of the head and neck district: Our experience with two cases.

INTRODUCTION: Apocrine adenocarcinoma (AA) is a rare gland cancer that appears in the elderly, especially males. Surgery is considered the first option for the management of this tumor. CASE PRESENTATION: We report two cases of AA that occurred at our Unit of Maxillofacial Surgery. Precisely a case of a woman with AA with a usual presence at the eyelid level and a case of a man with AA with an unusual presence at the neck level. DISCUSSION: This cancer generally arises in some specific areas of the body that present high concentrations of apocrine glands (such as in Case No.2). But it can also occur in less typical areas, such as the neck (such as in Case No.1). CONCLUSION: We discuss the surgical management of our cases: both based on our experience and literature data, we recommend extensive surgical excision.

Cutaneous apocrine gland neoplasia in 16 captive African wild dogs (Lycaon pictus).

This retrospective study documents the occurrence of single and multiple cutaneous apocrine gland tumours (CATs) on the dorsal midline of 16 captive African wild dogs (AWDs, Lycaon pictus) derived from 161 submissions to diagnostic laboratories in South Africa, France and Germany between 1997 and 2022. Animals included in the study came from zoological institutions in South Africa (n = 2), France (n = 5) and Germany (n = 1) and ranged from 5 to 14 years of age. Fifteen affected animals were female (94%) and one was male. CATs presented as raised, hairless, multilobular, grey firm masses, consistently located along the dorsal midline. Apart from a single cutaneous apocrine adenoma and a cystadenoma occurring concurrently with two non-cystic adenocarcinomas, neoplasms were consistent with malignant cutaneous apocrine adenocarcinomas with lymphatic spread and visceral metastases. Advanced age and female sex were identified as risk factors. A genetic component or association with the increasing use of GnRH agonist contraceptives was suspected but could not be established. This study highlights the need for close clinical monitoring of AWDs over the age of 5 years for the development of CATs along the dorsal midline and supports early surgical intervention. More research is needed to determine the role of inbreeding, endocrine changes and husbandry factors that may play a role in the development of CATs on the dorsal midline of AWDs.

Giant sebaceous nevus occupying half of the head associated with sebaceous carcinoma, apocrine adenocarcinoma, and basal cell carcinoma: A case report.

We herein report a 57-year-old man afflicted with multiple malignant tumors arising from a giant sebaceous nevus on his left parieto-temporal scalp and neck. The upper eyelid was resected in all layers, and the auricle was resected as well, leaving part of the external auricular cartilage; the parotid gland area was also resected over the parotid fascia. The lost part of the left eyebrow was reconstructed using an anterior forehead skin flap, and the residual defect was covered with skin graft and expanded scalp flap. This is probably the first case report of giant sebaceous nevus associated with three malignancies: sebaceous carcinoma, apocrine adenocarcinoma, and basal cell carcinoma.

Cervical lymphocele: A rare complication following salvage surgery for ocular adnexal apocrine adenocarcinoma. A case report and review of the literature.

BACKGROUND: An ocular adnexal apocrine adenocarcinoma (OAAA) is an extremely rare, but potentially aggressive and life-threatening tumor with ill-defined management based only on recommendations from a limited number of reported cases. The development of cervical lymphocele following neck dissection is a very rare complication, but one with well established methods for prevention and treatment. Here we describe a previously unreported case of salvage surgery including neck dissection for OAAA in addition to an emergence of cervical lymphocele. A literature review of current knowledge on both pathological conditions is included. METHODS AND RESULTS: A 58-year-old man suffering from OAAA, previously treated with multiple eye-sparing excisions and adjuvant proton therapy, underwent salvage surgery for locoregional recurrence of the tumor. A partial orbitectomy with orbital exenteration, primary reconstruction and left-sided neck dissection was performed. The procedure was complicated by a cervical lymphocele resolved after the surgical therapy. The patient remained disease-free during the one-year follow-up. CONCLUSION: OAAA is a locally aggressive tumor with potential to local or distant metastatic spread. Whole-body staging, regular clinico-radiological follow-up and stage-dependent therapy with surgery as the first-choice treatment is required. A cervical lymphocele as a complication of especially left-sided neck dissection is managed with a conservative or surgical therapy according to the level of lymph leakage, extent and localization of lesions, presence of local or systemic disorders and the period from primary surgery.

Metastatic cutaneous apocrine adenocarcinoma successfully treated with systemic anti-androgen therapy-A case report.

Primary cutaneous apocrine adenocarcinoma (PCAC) is an extremely rare neoplasm involving the sweat glands. Due to a lack of cases, there is no consensus for the systemic treatment of locally advanced or metastatic PCAC. Anti-androgen therapy may have activity in inoperable or metastatic PCAC with high androgen receptor (AR) expression.

What do you think of an unusual axillary mass?

Skin apocrine carcinoma is a rare malgnancy of epidermal adnexa, most frequent in axillary seat, where apocrine sweat gland are abundant, the neoplasm can arise in groin, anogenital, lips, eyelid, characterized by a plate or surface area of nodules hummocky. Etiology and incidence are not known. The prognosis is influenced by the risk of locoregional recurrence and metastatic evolution. We describe the case of 61-year-old man who presented a left axillary slow-growing mass since 2 years ago. The cutaneous biopsy objectified an apocrine adenocarcinoma. The paraclinic exams performed to detect primary breast were tumor negative, first step before confirming the diagnosis. Standard treatment is surgical excision with margins of 2 to 3cm for local tumor, for apocrine adenocarcinoma regional lymph node dissection if nodes were clinically positive is wide surgical excision. This kind of tumour is chemoresistant. In this case, adjuvant chemotherapy was indicated, before surgery to reduce tumoral volume. This case illustrates the importance clinicopathological correlation of skin cancer, particularly apocrine one. Clinical particularity and careful analyses histology helps diagnosis approach.

Apocrine gland adenocarcinoma in a persian cat remission with homeopathic treatment: case report

Apocrine gland carcinomas are rare malignant neoplasms that occur in cats. Available treatment is surgical, would lead to total ablation of the external acoustic meatus and usually recurrent [1,2].Methodology:In December 2020, a homeopathic consultation of the feline, male, Persian, 13 years old, a history of the disease was reported, which started in 2015, adding up to six recurrences of tumor processes in the eyelids and recent formation in the left ear canal.The result of the histopathological examination confirmed apocrine adenocarcinoma.Prior to homeopathic treatment, tumors recurred despite surgical interventions and prophylactic treatment with trichloracetic acid.However, remaining formations were noted in the eyelids and ear canal. It has a rounded blackened shape in the upper right eyelid measuring 0.4 cm and the lower 0.2 cm, round. In the left ear canal, around 1.6 cm, in addition to 4 points scattered in the ear folds. Homeopathic treatment was started for two months with Arsenicum album30 cH, twice a day; Carcinosinum 200 cH, once a day, and complex containing Avena sativa4 cH, Echinacea angustifolia4 cH, Conium maculatum6 cH, Thuja officinalis6 cH and Silicea terra6 cH, four times a day. Every two months, the clinical picturewas reassessed, potenciesand frequencies readjusted.In a few months,there was complete remission of the tumor, recovery of welfare, and improvement in mood and appetite. The free and informed consent term was signed. There was no recurrence of tumors until May2022. Conclusion:This study proved to be effective, documented with photos and exams. Approaching a rare case may provide a new therapeutic possibility. The credibility of quality homeopathic case reports has been increasing due to methodological requirements using tools developed in recent studies.

Apocrine Adenocarcinoma Occurring on the Chin.

We report a case of adenocarcinoma affecting the chin of a 48-year-old man. The tumor showed signs of apocrine differentiation and had infiltrated the muscle. The patient had no history or clinical evidence of breast cancer. We made a diagnosis of cutaneous apocrine adenocarcinoma. Apocrine adenocarcinoma rarely arises in areas with scarce apocrine glands. We reviewed the literature on apocrine adenocarcinoma of the face in areas other than the eyelids and auditory canal, where specialized apocrine glands are present.

Dose invasive apocrine adenocarcinoma has worse prognosis than invasive ductal carcinoma of breast: evidence from SEER database.

BACKGROUND: Invasive apocrine adenocarcinoma (AAC) of breast is a rare histopathological subtype of breast carcinomas. We aim to investigate the different characteristics and prognostic outcomes between AAC and invasive ductal carcinoma (IDC) of breast cancer. RESULTS: AAC patients presented with older ages, more aggressive behaviors, lower ER and PR proportions, higher HER2 amplification rates and less application of breast-conserving therapy and adjuvant chemotherapy compared to IDC patients. Long-term OS and DSS were both worse in ACC patients (p = 0.006, p = 0.012 respectively) than in IDC patients by Kaplan-Meier analysis. However, no significant difference was detected in DSS (p = 0.181) and OS (p = 0.116) between the matched two histological subtypes. Further subgroup analysis indicated that AJCC stage, ER status, PR status and HER2 status may be principal confounders for AAC prognosis. MATERIALS AND METHODS: With accession to the Surveillance, Epidemiology and End Result (SEER) database, a total of 260,596 patients met the eligibility criteria. Clinicopathological characteristics were compared between groups using Chi-square test. Univariate and multivariate analyses were applied to evaluate the overall survival (OS) and disease-specific survival (DSS). Subgroup analyses summarized the hazard ratio (HR) of AAC versus IDC using a forest plot. CONCLUSIONS: AAC had unique clinicopathological characteristics and it tended to be a more aggressive type than IDC. However, the worse prognosis was diminished after matching for demographic and clinicopathological factors. Deeper insights into AAC are in need to contribute to individualized and tailored therapy, which thereby may improve clinical management and outcomes.

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

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

Axillary apocrine adenocarcinoma in a young male suspected initially on fine-needle aspiration cytology.

Primary apocrine sweat gland adenocarcinomas are a rare entity, with only a few case reports so far. Many of these carcinomas are slow-growing with a high recurrence rate. A distinct cytological diagnosis can be made, and metastatic adenocarcinomas are always considered as a differential diagnosis on cytology. Our case was a 35-year-old male who presented with a discharging axillary sinus and swelling for the past 1 year. A clinical suspicion of tuberculous sinus was raised that however, remained unsupported by laboratory investigations. There was quite a high suspicion of apocrine adenocarcinoma on cytological examination that was confirmed by histopathology and immunohistochemistry. The patient was successfully treated with total excision and a wide margin. We report this case in view of its rarity and its occurrence in a 35-year-old young male, and emphasize that an initial cytological suspicion should be raised for primary apocrine adenocarcinoma in case of an axillary tumor, especially keeping in consideration the poor prognosis of the same and chances of early metastasis.