Primary Cutaneous Adenoid Cystic Carcinoma Research Articles

A Young Woman with a Cystic Mass in the External Auditory Canal

Introduction: Adenoid cystic carcinoma (ACC) is a malignancy that primarily affects the salivary glands. Primary cutaneous ACC (PCACC) is a rare variant that arises from ceruminous glands of the ear and other glands of apocrine origin. Case Presentation: We presented a case of a 26-year-old woman evaluated for a slow-growing mass in her ear for several years that was painful with bloody discharge. Physical exam demonstrated a subcutaneous nodule with overlying telangiectasias protruding from the external auditory canal. Excisional biopsy revealed a dermal proliferation of basaloid cells forming islands in a cribriform architecture. Discussion: PCACC grossly mimics other cysts and tumors, so diagnosis must be histologically confirmed. Basal cell carcinoma, mucinous carcinoma, and spiradenoma can mimic PCACC on microscopy, making differentiation important. PCACC is not associated with common risk factors for malignancy, thus it is important to include on the differential with any auricular tumor presenting as a pearly nodule with blue and purple hues. Despite treatment with wide local excision, postoperative radiation, and less commonly chemotherapy, recurrence rates are high. Conclusion: It is important to accurately diagnose PCACC, due to the need for long term follow up, as these tumors commonly recur decades later. This case contributes to the growing literature how PCACC can present and how the treatment will shape the trajectory of the patient’s follow up, as there is currently no consensus on definitive treatment.

Spiradenoma With Adenoid Cystic Carcinoma-like Changes: A Case Series of This Rare Variant With a Potential Diagnostic Pitfall.

Spiradenomas are benign cutaneous adnexal neoplasms with sweat gland differentiation that can manifest a broad spectrum of histomorphologic appearances. While they show a characteristic histopathologic phenotype and clinical management involves surgical excision with a low risk of recurrence, there have been unusual histopathologic variants of spiradenoma reported, including cases with adenoid cystic carcinoma (ACC)-like changes. Primary cutaneous ACC is a low-grade malignancy presenting as a subcutaneous mass with the potential for local invasion, perineural invasion, and high rates of local recurrence after excision. The diagnosis of spiradenomas with ACC-like features can be challenging, especially when only the ACC-like component is present for evaluation. A retrospective analysis of 21 cases of spiradenoma with ACC-like changes were obtained from large academic institutions, was performed, and summarized below. All cases showed background of conventional spiradenoma, and the ACC-like areas represented a component in all lesions. The percentage of ACC-like component ranged from 5% to 40% in all cases. The ACC-like component was multifocal and without pleomorphism, perineural and/or vascular invasion, necrosis, or increased mitotic activity. MYB translocation and protein expression was studied in 16 cases by fluorescence in situ hybridization, polymerase chain reaction, and immunohistochemistry. All studied cases were negative for MYB / NFIB , MYB L1, and MYB F by fluorescence in situ hybridization and polymerase chain reaction and 3 cases were positive for MYB expression by immunohistochemistry. Our study expands on spiradenomas with ACC-like features that ought to be considered in the differential diagnosis of cutaneous neoplasms such as primary cutaneous ACC. Our results indicate that a thorough histopathologic inspection and strict application of well-defined histologic criteria are necessary to support the diagnosis of this unusual histopathologic variant. These tumors can be difficult to diagnose, and awareness of their histomorphologic spectrum will facilitate definitive diagnosis and avoid misdiagnosis with other conditions.

44222 Primary Cutaneous Adenoid Cystic Carcinoma: Report of a Rare Tumor with Review of the Literature

44222 Primary Cutaneous Adenoid Cystic Carcinoma: Report of a Rare Tumor with Review of the Literature

Primary Cutaneous Adenoid Cystic Carcinoma Of The Chest Wall With Axillary Lymph Node Metastases And Its Management: A Case Report.

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare form of adenoid cystic carcinoma (ACC) arising commonly from the salivary gland. Less often they originate outside the head and neck region, with the scalp being the commonest cutaneous site in 40% of the cases. The presentation on the chest wall is rare, with no reports to date on axillary lymph node metastases. Here we report a case of a 65-year-old female with previously operated PCACC of the chest wall at a different center, showing uptake on positron emission tomography imaging at the site of surgical scar that w as inconclusive on needle biopsy metastasized to the axillary lymph node confirmed by needle biopsy managed with wide local excision, axillary lymph node dissection, and chest wall reconstruction with keystone island flap. The postoperative outcome was uneventful with no recurrence or axillary complications at one year's follow-up. She was advised to receive adjuvant radiotherapy; however, she refused. To conclude, though PCACC is rare, they can have an aggressive presentation, and a multidisciplinary approach is necessary for a better outcome.

Primary cutaneous adenoid cystic carcinoma of the scalp: dermatosurgical approach with favourable outcome.

Although described as early as 1975 as a distinct, rare form of cancer with diverse localization, primary cutaneous adenoid cystic carcinoma (PCACC) remains a mystery and challenge for both clinicians and pathologists. The clinical presentation cannot be clearly distinguished from amelanotic melanoma or intradermal nevus, Merkel cell carcinoma, trichofolliculoma, trichoepithelioma or other rare tumors of the adnexa, or dermatofibrosarcoma protuberans. The histopathological diagnosis requires not only careful evaluation of standard hematoxylin/eosin preparations, but also immunohistochemical staining with a number of markers such as epithelial membrane antigen (EMA), S-100, SOX-10, Ki- 67, CD-117 (c-kit), Vimentin, carcinoembryonic antigen (CEA), Ber-EP4 and many others. The surgical approach should consist of excision with margins between 1 and 2 cm, with the choice of margins depending upon the histopathological findings in the primary excisional specimen. We present a 31-year-old patient with an enlarging, amelanotic, plaque-like tumor of the scalp with a duration of no more than 18-24 months. Surgical treatment was performed within two surgical sessions with a total resection field of 1.3 cm. A good cosmetic result was achieved.

Primary cutaneous adenoid cystic carcinoma - An onerous task for clinicians and pathologists.

Adenoid cystic carcinoma (ACC) is an uncommon malignant neoplasm usually confined to the salivary glands, lungs, and breasts. Primary cutaneous adenoid cystic carcinoma (PCACC) is an extremely rare entity with solitary cases reported at sites away from the scalp and chest. Hence, one must follow the multidisciplinary approach to exclude any primary ACC elsewhere in the body. We report a rare case of PCACC arising from the skin of the left lower limb in a 55-year-old woman with a history of recurrent swelling, clinically diagnosed as a metastatic tumor.

Rare case of primary cutaneous adenoid cystic carcinoma of the abdomen: A case report

Adenoid cystic carcinoma is predominantly a tumor of the parotid glands and can sometimes be found in other glands. In most cases, skin location is usually a metastatic presentation and rarely a primary tumor. We describe the case of a 59-year-old female patient presenting with a 5-mm skin-colored nodule on the abdomen histologically compatible with a primary cutaneous adenoid cystic carcinoma. Extensive workup revealed no other primary source, nor evidence of metastatic disease; therefore, wide local excision was the preferred treatment given the low potential of recurrence. As this adnexal carcinoma is rare and its morphology non-specific clinically, we wanted to raise awareness of this entity and its management.

A Case of Primary Cutaneous Adenoid Cystic Carcinoma on the Axilla Showing Multiple Lung Metastasis

A Case of Primary Cutaneous Adenoid Cystic Carcinoma on the Axilla Showing Multiple Lung Metastasis

Primary cutaneous adenoid cystic carcinoma: a clinicopathologic, immunohistochemical, and fluorescence in-situ hybridisation study of 13 cases.

This study aimed to investigate the clinical, histological, immunohistochemical and chromosomal features of primary cutaneous adenoid cystic carcinoma (PCACC). We retrospectively analysed 13 cases identified on their clinicopathological features and performed fluorescence in-situ hybridisation (FISH) on six available cases. Head and neck (46.2%) were most commonly involved. The median age was 53 years, with a male predilection. Histologically, tumours were classified as grades 1 (eight), 2 (four) and 3 with high-grade transformation (HGT) (one). The HGT component was demonstrated as poorly differentiated carcinoma with multifocal necrosis and myoepithelial differentiation. Patients with one of the following factors: longest diameter of the lesion (≥ 1 cm), involvement of subcutaneous fat tissue and widely infiltrative border had a relatively higher rate of local recurrence, distant metastasis and death. Five of six cases were confirmed to have MYB translocation, while nuclear staining for MYB proto-oncogene, transcription factor (MYB) protein was found in four cases. During the follow-up (median = 64 months), two patients experienced local recurrences. One patient, who was classified as grade III PCACC with HGT, developed multiple metastases and died of disease. Another patient was alive with multiple metastases. This is the largest single-institution study, to our knowledge, of PCACC in an Asian population. We describe the first case of scalp PCACC with HGT, which is the only death case in our series. PCACC tends to recur locally and has metastatic potential. PCACC with HGT has a poor prognosis.

A Case Report of a Primary Cutaneous Adenoid Carcinoma: A Diagnostic and Management Challenge

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare glandular neoplasm that mimics benign lesions both clinically and histologically making it a diagnostic challenge. Although indolent, ACC has potential for local regional and distant metastasis and even after surgical excision, there is a high recurrence rate. This report outlines a case of a PPACC and reviews current therapeutic approaches.

Adenoid basal cell carcinoma: A rare variant and a diagnostic dilemma

Basal cell carcinoma (BCC) constitutes the most common malignancy of the skin, accounting around 70% of all skin malignancies, especially in sun-exposed areas. The adenoid variant is a very uncommon subtype which accounts for approximately 1.3% of all BCCs. A 65-year-old male presented with ulcer and swelling on the left side of the nose and cheek with rolled borders for the past 2 years. Based on the histopathology and immunohistochemistry, the diagnosis of the adenoid type of BCC was made. One should be aware of its close mimics such as primary cutaneous adenoid cystic carcinoma before diagnosing adenoid BCC as they differ in prognosis and treatment modalities.

Primary cutaneous adenoid cystic carcinoma : A case report

Primary cutaneous adenoid cystic carcinoma : A case report

Histopathology of primary cutaneous adenoid cystic carcinoma of the scrotum presenting with predominantly solid growth

Histopathology of primary cutaneous adenoid cystic carcinoma of the scrotum presenting with predominantly solid growth

Characteristics and Survival of Primary Cutaneous Adenoid Cystic Carcinoma of the Head and Neck

Behbahani, Sara MS; Wassef, David BS; Pinto, Jessica BS; Montovano, Margaret BS; Povolotskiy, Roman BS; Paskhover, Boris MD Author Information

Primary cutaneous adenoid cystic carcinoma: Characterizing US demographics, clinical course, and prognostic factors

To the Editor: Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare primary cutaneous malignancy, first described by Boggio1 in 1975. Histologically, PCACC presents with basophilic cells having adenoid or cribriform patterns due to true lumina within islands that are surrounded by modified myoepithelial cells and prominent basement membrane material.2,3 Given the somewhat conflicting data of indolent tumors as well as tumors with more aggressive behavior, we studied the incidence and survival patterns for patients with PCACC in the United States.

Analysis of Characteristics and Survival of Primary Cutaneous Adenoid Cystic Carcinoma of the Head and Neck.

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare skin tumor. The head and neck (H&N) is the most common anatomical location. Due to limited published cases, its clinical course and management are not well understood. The National Cancer Database (NCDB) was queried for all cases of H&N PCACC diagnosed from 2004 to 2016. Kaplan-Meier (KM) and Cox proportional hazards models were used to determine clinicopathological and treatment factors associated with survival outcomes. A total of 201 cases were analyzed. The average age of diagnosis was 57.7 years (± SD 15.8). There was a female predilection (57.7%; P < .05) with the most common primary site being the ear (58.2%). The average tumor size was 15.9 mm in diameter. The most common treatment was surgery alone (51.7%) with wide local excision being the common surgery performed (36.3%). 5-year and 10-year OS were 87.0% and 76.0%, respectively. A total of 65.8% of cases were localized (Stage I and II). KM analysis indicated that gender, age, insurance status, Charlson-Deyo Comobordity Score, and stage were significant predictors of OS (P < .05). Cox proportional hazards analysis revealed that patients with both private (HR 0.11, 95%CI [0.019-0.670]; P = .02) and government (HR 0.12, 95%CI [0.019-0.972]; P = .03) health insurance had a significantly decreased hazard of death than patients who were uninsured. Increasing age was associated with an increased hazard of death (HR 1.06, 95%CI [1.016-1.110]; P = .01). This study represents the largest cohort of H&N PCACC studied to date and provides important clinicopathologic information for this rare tumor. Additionally, our results emphasize the importance of health insurance as an independent predictor of survival in PCACC.

Primary cutaneous adenoid cystic carcinoma of the abdomen: a rare entity

Adenoid cystic carcinoma is a rare neoplasm that arises from secretory glands, most frequently from the salivary glands. Primary cutaneous adenoid cystic carcinoma is microscopically identical to adenoid cystic carcinoma developing at other tissues. Therefore, differentiating between a primary cutaneous adenoid cystic carcinoma and an extracutaneous adenoid cystic carcinoma with cutaneous metastases is pivotal to determine its prognosis and management. We describe a case of primary cutaneous adenoid cystic carcinoma on the abdomen that was successfully treated with wide excision.

Primary Cutaneous Adenoid Cystic Carcinoma of the Lower Limb: Rare Tumor in a Rare Location. A Case Report and Brief Review of Literature

Abstract Adenoid Cystic Carcinoma (ACC) is a rare entity with a predilection site at the salivary glands. Cutaneous localization is rather exceptional. Despite its rarity, it has a very distinctive histology consisting of cribriform epithelial structures which form pseudoglandular spaces giving a characteristic ‘swiss cheese’ appearance. Those pseudoglandular areas contain basement membrane-like material. The tumor has also the tendency for perineural invasion, that is responsible for its aggressiveness. We describe a case of a 60-year old male presenting with a pretibial asymptomatic pigmented dermal nodule on the lower leg. Histological examination was consistent with the characteristics of an ACC. After thorough examination we excluded localization elsewhere in the body and confirmed the primary cutaneous origin. In this case report we also briefly refer to the histogenesis, the morphological and molecular profile, the differential diagnosis, the therapeutic options and the management of the Primary Cutaneous Adenoid Cystic Carcinomas (PCACCs). Keywords Primary cutaneous adenoid cystic carcinoma; Skin cancer; Tibia

Primary Cutaneous Adenoid Cystic Carcinoma

Adenoid cystic carcinoma is a malignant tumor usually localized in the salivary glands. It can also develop in serous glands such as the breast, main bronchi, uterine cervix, Bartholin's gland of the vulva, prostate gland, and external auditory canal. It is more common in the white race. Most of the primary cutaneous adenoid cystic carcinoma cases occur on the scalp. Despite the high rate of metastasis, longterm survival can be observed because the progression of the disease is slow. Distant organ metastases such as lungs, bone, liver and brain have also been reported in the literature at 40%. Its treatment is wide excision of the lesion and demonstration of tumor negative margin in pathological sampling. In this case report, we wanted to present surgical and oncological treatment to a 65-year-old female patient with lung metastasis diagnosed with adenoid cystic carcinoma on the scalp.

Primary Cutaneous Adenoid Cystic Carcinoma: A Case Report and Literature Review

Primary Cutaneous Adenoid Cystic Carcinoma: A Case Report and Literature Review