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.

BACKGROUND: High-grade transformation Adenoid cystic carcinoma (HGT-AdCC) of the parotid gland is a rare transformation noted in slow growing low grade AdCC. Perineural invasion and spread is an important feature of this tumor. Temporal bone involvement is rare. A total of only 10 cases of HGT-AdCC in parotid gland has been reported in literature so far predominantly in the elderly with peak incidence in 5th-6th decade.
METHODS: We present a young lady of HGT-AdCC of right parotid gland with temporal bone involvement in the form of isolated perineural invasion (PNI) of facial nerve till the tympanic segment. She underwent right radical parotidectomy with modified radical neck dissection with modified lateral temporal bone resection and pectoralis major myocutaneous flap reconstruction. Histopathological examination revealed both low- and high-grade areas. Sections from facial nerve showed tumor invasion.
CONCLUSIONS: The radiological features of isolated perineural spread in intratympanic part of facial nerve can be easily missed if not specifically looked for. Every attempt should be made preoperatively and intraoperatively to determine the complete extent of the tumor for adequate disease clearance. A combined clinico-radiological approach aided by histopathology examination helps in early detection of this carcinoma and in better patient management.

BACKGROUND: Breast adenoid cystic carcinoma (AdCC) is a rare invasive carcinoma composed of epithelial and myoepithelial cells. Microglandular adenosis (MGA) is a rare benign proliferative lesion consisting of small, uniform, and round glands formed by a single layer of epithelial cells and basement membrane without a myoepithelial cell layer. MGA may progress to atypical MGA and carcinoma arising in MGA. Among various invasive carcinomas from MGA, AdCC has been rarely reported. Here, we report a case of AdCC arising in MGA.
METHODS: A 59-year-old woman was diagnosed with a newly developed density on a routine mammogram. The density was similar to or slightly lower than that of the breast parenchyma. Sonography showed an irregular mass with a slightly higher echo than that of fat. Magnetic resonance imaging showed an irregular mass with a similar T1 signal intensity and a slightly higher T2 signal intensity compared to muscles or the breast parenchyma. The lesion showed heterogeneous internal enhancement with an initially slow and delayed persistent enhancing pattern. Microscopically, the tumor was composed of invasive AdCC, in situ AdCC, and MGA. AdCC is composed of basaloid and ductal epithelial cells forming cribriform or solid sheets, or haphazardly scattered small cribriform or tubular glands. MGA showed small glands with a single epithelial lining and retained lumen. S-100 staining was strongly positive in MGA area. The patient underwent breast-conserving surgery with sentinel lymph node biopsy.
CONCLUSIONS: Breast AdCC arising in MGA showed unique imaging findings that was different from usual invasive cancer.

BACKGROUND: Adenoid cystic carcinoma (ACC) is an uncommon tumor of the breast, accounting for approximately 0.1% to 1% of all breast cancers. It is characterized by rare lymph node involvement and distant metastasis, and associated with a favorable prognosis with excellent survival, despite its triple-negative status. In the current state of knowledge, results of breast-conserving treatment with postoperative radiotherapy seem to be equivalent to mastectomy alone, with respect to survival for ACC of the breast. Due to its rarity, there is no consensus on optimal treatment for patients with ACC. Otherwise, the role of chemotherapy and hormonal therapy remains controversial. Further clinical studies are required to compare treatment options for ACC. But, a long-term follow-up is very important and mandatory for affected patients, due to the late onset of local relapse and occurrence of distant metastasis.
METHODS: Here, we report the case of a patient who presented with a palpable breast mass in the left breast that turned out to be an ACC of the breast.

Adenoid cystic carcinoma is a malignant neoplasm most commonly originating in salivary glands of head and neck region. Among intra oral adenoid cystic carcinoma, buccal mucosa is among the rarest sites. We report a case of adenoid cystic of buccal mucosa in a 40-year old female. We have discussed the clinical features, histopathology, diagnosis and treatment along with a brief review of the relevant literature. Although the buccal mucosa is an uncommon site for adenoid cystic carcinoma, the relatively indolent growth pattern of this case and its location which is rather atypical for this type of salivary gland malignancy primarily warrants the necessity behind reporting of this case. Secondly, adenoid cystic carcinoma should be considered in the differential diagnosis of mass of buccal mucosa. It is important to identify such cases rather early and surgical removal with adequate margins is the treatment of choice .

BACKGROUND: Adenoid cystic carcinoma (ACC) of the lacrimal gland is rare but with high recurrence rates and late metastases. They occasionally metastasise via haematogenous spread to lungs, brain and bone. Liver is a rare site of metastasis and is usually present with disseminated disease.
METHODS: A 42 year old lady, a known case of adenoid cystic carcinoma of the lacrimal gland who had been operated 4 years ago for the same followed by chemotherapy and radiotherapy. She was detected with large hepatic and bilateral multiple pulmonary metastases. A right hepatectomy was done. After six months when her general condition improved we did a staged pulmonary metastatectomy. Histology confirmed the diagnosis. She is presently doing well with no recurrence since 18 months.
CONCLUSIONS: Usually asymptomatic, the commonest symptom is pain. It is a locally invasive disease with a poor prognosis if detected late. Surgery is the only proven therapy. Isolated hepatic metastases being managed surgically has been reported. However, extensive, staged, surgical resection of hepatic and pulmonary metastases has not been reported.
CONCLUSIONS: Lacrimal ACC is an aggressive tumour with known late metastases with the liver being affected seldomly. Our case highlights that even disseminated metastases to the liver and the lungs from lacrimal ACC can be managed surgically with a good outcome. To our knowledge, no such report with distant metastases to the liver and the lung along with their successful surgical management has been reported.

Introduction Adenoid cystic carcinoma (ACC) is one of the most frequent malignant salivary gland tumors, which commonly affects the minor salivary glands of the mouth and is rare in the nose and paranasal sinuses. In the maxillary sinus, ACC can mimic inflammatory diseases and has a poor prognosis. Objective To report a case of a 50-year-old man with ACC of the maxillary sinus whose clinical findings in the alveolar ridge mimicked an oroantral fistula. Case Report An excisional biopsy was performed and histopathologic analysis revealed ACC. Lung metastases and residual tumor in the maxillary sinus were detected by imaging methods. In view of the poor general health of the patient, no new surgical intervention was performed and he was only treated by radiotherapy and follow-up. Conclusion Although rare in the maxillary sinus, ACC should be included in the differential diagnosis of lesions affecting this site.

Adenoid cystic carcinomas are deceptive malignancies that show slow growth and local invasion with recurrences seen many years after diagnosis. Upto 50% of these tumors occur in the intraoral minor salivary glands usually in the hard palate. Buccal mucosal tumors are relatively rare. We determined the incidence of buccal mucosal adenoid cystic carcinoma by reviewing the number of reported cases in the literature. This is the first article to analyze the occurrence of adenoid cystic carcinomas in the buccal mucosa through a review of 41 articles. Our review revealed 178 buccal mucosal adenoid cystic carcinomas among a total of 2,280 reported cases. We present a case of adenoid cystic carcinoma occurring in the left buccal mucosa of a 45-year-old female.

Adenoid cystic carcinoma (ACC) constitutes about 4% of salivary epithelial tumors and is the second common malignant epithelial salivary gland tumor involving both the major and minor salivary glands. High grade transformation in ACC is a recently recognized entity with only a few cases reported in literature. We report the first case of ACC with high grade transformation involving the parotid. A 54-year-old man with a history of right parotid painful swelling from 1.5 years presented with recent increase in size of the swelling and facial paresis of 2 months duration. The locally invasive salivary neoplasm was composed predominantly of an undifferentiated carcinoma with foci of conventional ACC occupying less than 10% of tumor area. Immunohistochemical study of the undifferentiated component as compared to conventional ACC showed greater over-expression of p53 and Ki-67. Her-2/Neu was negative in both components. Recognition of occasional clusters of basaloid cells and hyaline globules in association with larger poorly differentiated malignant cell population in aspiration smears can help in cytological diagnosis. The acquisition of high proliferation index and over-expression of p53 may be the probable factors involved in the pathogenesis of high grade transformation in a conventional ACC.