European Journal of Cardiovascular Medicine

Adenoid Cystic Carcinoma (ACC) is a rare tumor entity and comprises about 1% of all malignant tumor of the oral and maxillofacial region1. In addition to the salivary glands, it affects the lacrimal glands, ceruminous glands and occasionally the excretory glands of the female genital tract. The tumor is typically slow growing compared to other carcinomas and has a tendency for perineural invasion as well as hematogenous spread to distant organs and is most seen in the elderly2. We are reporting a case of Adenoid cystic carcinoma presenting as a large Ulceroproliferative growth over right cheek in a 64-year-old female.

A 64-year-old lady came to our OPD with a large painful Ulceroproliferative growth over right cheek since last one and half years. Initially it was smaller and gradually progressing and attained present size. Initially it was associated with dull aching, sudden onset pain. Clinical examination revealed a large Ulceroproliferative growth over right cheek measuring 05x04x05 cm involving the right buccal mucosa and right upper gingivobuccal sulcus (Fig. 1). It was slightly tender on touch, rough surfaced with everted and rolled out margins along with indurated base. The provisional diagnosis was given as Squamous cell carcinoma. Differential diagnoses were Sarcoma and salivary gland neoplasms. CT Scan of paranasal sinuses with contrast showed an ulceroproliferative growth involving the right buccal mucosa, right upper gingivobuccal sulcus, and gingiva of the right maxillary alveolus, suggestive of neoplastic etiology. The major salivary glands were normal in morphology and size. FNAC was suggested and it showed multiple variable sized globular, spherical, and tubular homogenous acellular magenta colored matrix material surrounded by cells and clusters and dissociated small cells with scant cytoplasm with round monomorphic hyperchromatic nuclei with coarse chromatin (Fig.2). Cytomorphological features were suggestive of Adenoid cystic carcinoma. Wide local excision was done, and specimen sent for histopathology. Histopathological examination showed an invasive tumor arranged in predominantly variably sized nests with cribriform pattern and few small solid nests and occasional tubules. Tumor cells have mildly enlarged nucleus with vesicular to clumped chromatin, tiny nucleolus admixed with cells with elongated hyperchromatic nuclei (Fig. 3&4). Tumor was infiltrating into overlying skin with ulceration. Perineural invasion was present. Thus, histopathological examination confirmed the cytological diagnosis of Adenoid cystic carcinoma (cribriform pattern). Fig 1. Ulceroproliferative growth over right cheek. Fig 2. Cytology smear showing globular homogenous acellular magenta colored matrix material surrounded by cells (x40, Giemsa stain) Fig 3. Section showing invasive tumor arranged in predominantly variably sized nests with cribriform pattern. (x20, H&E stain) Fig 4. Section showing invasive tumor arranged in cribriform pattern. (x40, H&E stain)

Adenoid cystic carcinoma (ACC) represents 3-5% of head and neck malignancies3 and 5-15% of sinonasal malignancies. It is characterized by an indolent but aggressive clinical course, the presence of early perineural invasion, frequent local recurrence, and a high rate of delayed distant metastasis.

n his intriguing work, “History of Surgery of the Upper Jaw,” Stell PM stated, “It is often said that adenoid cystic carcinoma was first described by Billroth in 1859 under the term Zylindrome4. The tumor was originally described by two Frenchmen, Robin and Laboulbene, in 1853 as "tumeur hétérogène." While the histopathological term "cylindroma" has been used for many years due to the characteristic appearance of cylindrical clusters of epithelial and secretory cells surrounded by a hyaline stroma, the term "adenoid cystic carcinoma" was later introduced by Ewing in 19545.

One of the most outstanding features of ACC, regardless of its site of origin, is its marked tendency to invade nerves. ACC is generally recognized for its frequent and often silent distant metastases. They are rare at presentation, but at 10 years they account for approximately 30% to 40%. The commonest sites are lung, bone, liver and brain6. Imaging with either CT or MRI is mandatory when there is the clinical suspicion of malignancy. FNAC has become a commonly performed diagnostic test in the initial evaluation of a major salivary glands mass. The advantage of this technique is that it can be performed in the outpatient setting with a low risk of complications. However, FNAC has a low diagnostic potential for tumor typing and grading. In a study of 160 patients of adenoid cystic carcinoma by Fordice et al., disease-specific survival was 89% at five years but only 40% at 15 years. ACCs generally carry a favorable prognosis with a 5-year survival rate of around 75%, but the 10-year survival rate drops acutely to 20%. Features associated with poor prognosis include the size of >4 cm of the primary tumor and a solid subtype histological pattern. Other features like perineural invasion and bone involvement are also associated with an unfavorable outcome and increased mortality7.

Initial treatment for ACC is often surgery with or without radiation therapy, but most patients eventually experience tumor recurrence and/or metastasis. Chemotherapy is administered to control advanced, unresectable, recurrent and/or metastatic ACC, but a standard-of-care chemotherapy protocol is yet to be determined8.

Adenoid cystic carcinomas are unpredictable, slow growing tumors that tend to invade perineural spaces. Recurrence is very common, and 50% or more eventually disseminate to distant sites including bone, liver, and brain, sometimes decades after attempted removal. Adenoid cystic carcinomas arising in the minor salivary glands tend to have a poorer prognosis than those arising in the parotid glands. This case of ACC presenting as a large ulceroproliferative mass on the cheek is a reminder of the tumor’s capacity for diverse clinical manifestations. Early and accurate diagnosis, followed by appropriate surgical management, is crucial in optimizing outcomes. The role of various diagnostic investigations like CT scan, FNAC and Biopsy are discussed here. Combination of Surgery and radiotherapy remains the treatment of choice as in our case.