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Myoepithelial carcinoma (MECA) is a rare salivary gland (SG) neoplasm (0.1–0.45% of all SG tumors) that often presents with bland cytomorphology and can be misclassified as cellular pleomorphic adenoma (PA) or myoepithelioma. This is particularly challenging in MECA ex-PA cases, especially if tumor shows minimal to no capsular invasion. We report a rare case of a 76-year-old female; history of left superficial parotidectomy with diagnosis (outside hospital) of cellular PA, who re-presented 9 months post surgery with enlarging left parotid mass, neck lymphadenopathy and facial nerve deficits. FNAB of parotid and neck lymph node revealed cellular aspirates with loosely cohesive clusters of myoepithelial cells with occasional chondromyxoid stroma. Prior resection slides were reviewed, and diagnosis of MECA ex-PA was made. Patient underwent left radical parotidectomy, selective neck dissection, with facial nerve sacrifice (due to extensive encasing by tumor). Histology showed a multinodular tumor with pushing borders, zonal arrangement comprising of a hypocellular, necrotic/myxoid center, and a peripheral rim of myoepithelial cells, confirmed by positive S100, and p63. Tumor extensively infiltrated peri parotid soft tissues with multiple foci of lymphovascular and perineural invasion; and metastatic neck lymph nodes. Next generation sequencing revealed a novel TERT promoter mutation (c.-124C > T), not usually described in SG neoplasms. Further, PD-L1 immunohistochemistry showed positive expression, making patient eligible for anti-PDL-1 immunotherapy. This case highlights importance of recognizing the subtle malignant features of MECA in distinguishing it from benign mimics like PA. In addition, presence of TERT mutation opens a new arena for future research to explore potential treatment targets.

Salivary gland tumors are relatively uncommon with most being benign. When diagnosed the most common benign and malignant tumors are pleomorphic adenoma and mucoepidermoid carcinoma (MEC), respectively. However, not uncommonly, it is difficult to differentiate between the histopathological entities, leading to a diagnostic dilemma that can impact a patient’s treatment and prognosis. A 24-year-old woman presented with a three-year history of asymptomatic left-sided facial swelling. She denied any prior history of head and neck radiation. There was no history of alcohol consumption or smoking exposure and there was no personal or family history of head and neck cancers. Additionally, she did not have any known occupational or environmental exposures. Due to the chronicity and painless nature of this facial mass, our patient did not pursue evaluation initially. Subsequently, she experienced an increase in size and pain for a few months exacerbated by swallowing. She had no other symptoms. On physical examination, a 3 x 3 cm left parotid gland mass was noted. There was no associated head or neck lymphadenopathy and compression of the left facial mass did not elicit secretions from the opening of Stensen’s duct. Due to the rapid increase in size, she was sent for CT neck/soft tissue with contrast which confirmed a 3.56 x 2.67 cm solid nodule within the superficial portion of the left parotid gland. This was followed by an MRI orbit/face/neck with and without contrast, for further delineation, which demonstrated a 4 x 3.7 x 3 cm complex heterogeneous mass within the superficial left parotid gland. Thereafter the patient underwent an uncomplicated ultrasound-guided biopsy of the parotid mass. The histopathological appraisal concluded that this was a cellular pleomorphic adenoma, with mucinous and squamous metaplasia with reactive lymph nodes. Due to the new rapid increase in size and intense painful nature of this tumor, nerve-sparing left parotidectomy, fat grafting and reconstruction were completed. Cellular pleomorphic adenomas are benign low-grade neoplasms, typified as biphasic with both epithelial and myoepithelial components. However, they have increased cellularity and focally increased mitotic activity, not advanced enough to qualify as malignant. The presence of mucinous and squamous metaplasia is of diagnostic interest as it makes diagnosis on fine-needle aspiration (FNA) morphologically challenging. These findings are typical of MEC and on FNA can be misleading in the setting of a pleomorphic adenoma. However, on histopathological evaluation of the gross specimen along with immunohistochemical staining the diagnosis is made much easier. A diagnosis of MEC would have potentially required neck dissection and adjuvant therapy with a potential increased risk of morbidity and mortality. This case emphasises the importance of an adequate tissue biopsy in regards to parotid gland tumors to optimise a patient's care plan.

Membranous basal cell adenoma (MBCA) is a rare benign salivary gland neoplasm. It is difficult to diagnose MBCA based on fine-needle aspiration (FNA) cytology due to rare reporting of its FNA cytology and overlapping of its FNA cytologic features with some benign and malignant entities. We present a case of MBCA in a 67-year-old female that was originally misinterpreted as adenoid cystic carcinoma (ACC) on FNA cytology. The FNA smears showed numerous uniform small basaloid epithelial cells with round or oval nuclei and inconspicuous nucleoli, and scant cytoplasm. The basaloid cells surround acellular, dense, homogenous material or are surrounded by acellular or paucicellular dense homogeneous material possibly containing bland spindle cells. The basaloid cells are present in variably sized three-dimensional clusters, acini, or sheets with variable cohesion. The dense homogenous material surrounded by basaloid cells may be interconnected. High power magnification reveals the homogeneous material to have a fibrillar texture. The edges of dense homogenous materials were well-demarcated. We describe the diagnostic pitfalls of FNA for MBCA, particularly versus ACC, basal cell adenoma, cellular pleomorphic adenoma, myoepithelioma, basal cell adenocarcinoma, and basaloid squamous cell carcinoma in hope of improving clinical management and patient treatment.

Myoepithelial carcinoma (MC) is rare malignant salivary gland neoplasm and its cytologic features have been rarely described in the literature. Furthermore, MC shows varied cell types and patterns leading to the wide range of differential diagnosis on cytology. Histopathology and immunohistochemistry (IHC) are necessary to make a definite diagnosis. A 37-year-old female presented with painless, progressive swelling in the infra-auricular region since 2 years. Fine-needle aspiration cytology was performed and cytological possibilities of cellular pleomorphic adenoma and myoepithelial cell neoplasm were rendered and patient was advised excision and histopathologic examination for final diagnosis and subtyping. Final diagnosis of MC was made on hematoxylin and eosin sections and IHC. MC is rare malignant salivary gland tumor showing a clinic-pathologic diversity. The cytological features of MC are diverse and may lack overt feature of malignancy. Pathologists should be aware of this entity while evaluating cytological smears of salivary gland mass.

Basal cell adenoma (BCA) is a rare neoplasm which is one of the basaloid tumors of salivary gland. Basaloid tumors are the most difficult problem in salivary gland fine needle aspiration cytology (FNAC). There are various benign and malignant tumors such as; cellular pleomorphic adenoma, basal cell adenocarcinoma, adenoid cystic carcinoma, metastatic basal cell carcinoma, metastatic basaloid squamous carcinoma and small cell carcinoma in differential diagnosis. We present a case of BCA, membranous type in a 39-year-old female with right submandibular swelling misinterpreted as adenoid cystic carcinoma (ACC) on FNAC.

Basal cell adenoma (BCA) is a rare benign basaloid neoplasm of the salivary gland. There are four histopathological types of BCA: solid, tubular, trabecular, and membranous. It is known that focal squamous metaplasia may be seen in some BCAs, but it is rare to see extensive squamous metaplasia, especially with cellular atypia. Here, a 25-year-old male with right parotid swelling is presented. Ultrasonography revealed a 2-cm well defined mass in his parotid gland. Fine-needle aspiration (FNA), performed prior to surgical excision, showed a highly cellular tumor composed of basaloid cells, forming small duct-like or tubular structures containing basement membrane-like material, as well as squamous cells with hyperchromatic, enlarged, pleomorphic, and bizzare nuclei. We made a cytopathological diagnosis of "basaloid neoplasm" and also reported that the differential diagnosis included BCA, cellular pleomorphic adenoma, basal cell adenocarcinoma, and carcinoma ex pleomorphic adenoma. The patient underwent total parotidectomy. Both frozen and permanent sections showed a BCA with membranous, tubulotrabecular pattern, and extensive squamous metaplasia. Some of the squamous cells showed significant nuclear hyperchromasia, enlargement, and pleomorphism. As far as we know, this is the first case of BCA with extensive squamous metaplasia and prominent cellular atypia. This case has been presented to show that squamous metaplastic cells with hyperchromatic, enlarged, bizarre, and pleomorphic nuclei can be seen on FNA smears of a benign tumor like BCA. Also, the differential diagnosis of BCA of the salivary gland is discussed here.

Papillary microcarcinoma may present with clinical symptoms, most commonly jugulodigastric and pretracheal lymphadenopathy with or without palpable thyroid nodules. Isolated submandibular metastases are rare. We are presenting here a case report of 40-year old lady with an isolated submandibular gland metastasis arising from a papillary thyroid carcinoma. Submandibular gland excision of right side was done based on the fine needle aspiration cytology (FNAC) which suggested features of Basal cell adenoma vs cellular pleomorphic adenoma. USG neck s/o 3×3.5×2.8 cm sized hypoechoic lesion right submandibular. Histopathologic examination of the specimen suggested a metastatic papillary carcinoma. Total thyroidectomy was done, no other site of metastasis was found on radio-iodine scanning.

Polymorphous low grade adenocarcinoma (PLGA) is a tumor of minor salivary glands that exhibits considerable morphologic overlap with adenoid cystic carcinoma and cellular pleomorphic adenoma, especially in small biopsy specimens. Unlike these other tumor types. PLGAs do not harbor a myoepithelial component, yet their frequent positivity for p63 diminishes the usefulness of this particular myoepithelial marker as a discriminating immunostain. p40 is an antibody that recognizes ΔNp63, a p63 isoform that is more specific for true myoepithelial differentiation. As such, p40 immunostaining could help distinguish PLGAs from adenoid cystic carcinomas and pleomorphic adenomas. In this study, p63 and p40 immunohistochemistry was performed on paraffin embedded, formalin fixed tissue from 11 PLGAs, 101 adenoid cystic carcinomas, and 31 pleomorphic adenomas. All 11 PLGAs (100 %) were positive for p63 but completely negative for p40. Among adenoid cystic carcinomas, 91 of 101 (90 %) were positive for p63 and 90/101 (89 %) were positive for p40. The single discordant p63+/p40− adenoid cystic carcinoma exhibited solid architecture and high grade features not typically seen in PLGA. Among pleomorphic adenomas, 21/31 (68 %) were positive for p63 and 13/31 (42 %) were positive for p40. For the pleomorphic adenomas, the discordant p63+/p40− staining pattern was seen only in the overtly mesenchymal chondromyxoid stroma. The cellular epithelial component of the pleomorphic adenomas demonstrated concordant p63+/p40+ or p63−/p40− immunophenotypes. PLGA consistently exhibits a p63+/p40− immunophenotype that can help distinguish it from adenoid cystic carcinoma and cellular pleomorphic adenoma, tumors that characteristically demonstrate concordant p63 and p40 immunostaining patterns. A p63/p40 immunohistochemical panel can provide a valuable tool for making the distinction between these morphologically similar but clinically divergent entities.

OBJECTIVE: To retrospectively assess the diagnostic accuracy, cytologic features and pitfalls of Thin Prep® (TP) (Cytyc Corporation, Marlborough, Massachusetts, U.S.A.) versus conventional (smear) preparation (CP) in salivary gland fine needle aspiration biopsies (FNABs) and second, to evaluate the reproducibility of the cytomorphologic criteria used in the evaluation of FNABs prepared by CP versus TP. STUDY DESIGN: All salivary gland fine needle aspiration biopsies (SGFNABs) between January 1996 and June 1999 were retrieved from the cytology files of the University of Michigan Hospital. Histologic correlation was identified when available. Two cytopathologists reevaluated the slides for artifacts, cellular preservation, background material, cellularity, and cytoplasmic and nuclear details. RESULTS: Seventy-four of the 134 (55%) cases identified had histologic follow-up. Fifty (68%) cases were processed by TP and 24 (32%) by CP. FNAB processed by TP and CP correctly identified malignancy in 14 and 9 cases, respectively. There were three (4%) false negative cases. These included two acinic cell carcinomas and one mucoepidermoid carcinoma. There were 37 true negative cases (24 TP and 13 CP) and one false positive case of cellular pleomorphic adenoma (cytologic interpretation, mucoepidermoid carcinoma). All discrepant cases were processed using the TP method. The overall specificity and sensitivity were 98% and 88%, respectively. However, specificity and sensitivity for TP-processed SGFNABs were 96% and 82% as compared to a 100% specificity and sensitivity for CP. Additionally, there were 10 (14%) nondiagnostic cases, 8 of which were processed by TP. Cytologic artifacts associated with TP included diminished/distorted extracellular and stromal elements, cellular shrinkage and tissue fragmentation CONCLUSION: The diagnostic accuracy of TP-processed SGFNABs approaches that of the CP. However, there are several artifacts that may lead to erroneous diagnoses. Additional studies, that depend on real-life clinical samples processed by TP are suggested to modify current diagnostic criteria.

Salivary gland tumours are rare in children and when they do arise, they preferentially affect major salivary glands with sporadic incidence in minor salivary glands. The mucosa of the cheek is an uncommon site of occurrence for intraoral pleomorphic adenoma and most of these cases have been reported in adults. Histologically, it shows a highly variable morphology because of interplay between epithelial and mesenchymal (myxoid, hyaline, chondroid, osseous) elements which arise from same cell clone, which may be a myoepithelial or ductal reserve cell. Here we report a rare case of juvenile pleomorphic adenoma of the cheek in a 12-year-old girl with a predominant epithelial component histologically. Relevant studies are discussed with a focus on its cytology and cytogenetics.

Encapsulated Minimally Invasive Epimyoepithelial Carcinoma of Submandibular Gland - A New Entity?? With Review of Literature Epimyoepithelial carcinoma (EMC) is a rare low-grade salivary gland malignancy comprising of 1% of all the salivary glands tumors. EMC is a rare tumor when involving submandibular gland (8.6%) as compared to parotid gland (62.1%). A thorough pubmed literature search retrieved only 17 cases of EMC involving submandibular gland since 1972. EMC can present in classical pattern characterised by biphasic cell population with inner layer of cuboidal to columnar cells and outer layer of myoepithelial cells. Encapsulated minimally invasive epimyoepithelial carcinoma is a recently coined terminology by Seethala et al. To the best of our knowledge, this is the first case of encapsulated minimally invasive EMC of submandibular salivary gland discussed in the literature. The present paper highlights the review of literature of EMC involving submandibular salivary gland, histomorphological spectrum and diagnostic differentiation from other salivary gland tumors.

Pleomorphic adenoma, the most common salivary gland tumor, accounts for 54 to 65% of all salivary gland neoplasm and 80% of the benign salivary gland tumors. Most frequently it affects the parotid gland, followed by the submandibular and the minor salivary glands. Microscopically, mucous, sebaceous, oncocytic and squamous metaplasia sometimes with the formation of keratin cyst, may be present. Extensive squamous metaplasia can be mistaken for malignancy, including mucoepidermoid carcinoma and squamous cell carcinoma. Here, we present an unusual case of pleomorphic adenoma with presence of extensive squamous metaplasia, keratin cyst formations and dense cluster of basaloid cells with spherical hyaline globules giving rise to suspicion of mucoepidermoid and adenoid cystic carcinoma.

The cytologic features of five cases of epithelial-myoepithelial carcinoma arising in major salivary glands (four parotid and one submandibular gland) are presented. All cases were initially diagnosed as low-grade malignancies. The smears were generally cellular, with no specific architectural pattern. Single cells and naked nuclei were prominent in all cases. A biphasic epithelial (small-cell) and myoepithelial (large/clear-cell) pattern was identified readily in two cases and with difficulty in one case. The biphasic pattern may be subtle or absent since the clear cells have a fragile cytoplasm and often appear as naked nuclei. The differential diagnosis includes adenoid cystic carcinoma, polymorphous low-grade adenocarcinoma, and cellular pleomorphic adenoma.

Basaloid tumors of the salivary glands are a heterogeneous group of benign and malignant lesions characterized by small tumor cells with round or ovoid nuclei surrounded by a thin rim of cytoplasm. Primary salivary gland tumors with this predominant morphology include basal cell adenoma, basal cell adenocarcinoma, cellular pleomorphic adenoma, adenoid cystic carcinoma, and small cell undifferentiated carcinoma. Certain metastatic lesions and nonepithelial neoplasms can also demonstrate a basaloid appearance. Histologic diagnosis based on resected tumors is usually straightforward when the architecture can be adequately assessed. However, in limited biopsies and particularly in cytologic samples, the evaluation can be quite challenging. A systematic approach aided by immunohistochemistry is essential to arrive at an accurate diagnosis.

Objective To study the spectrum of cytologic changes seen in cellular pleomorphic adenoma (CPA) and to determine features that would allow a more specific diagnosis. Study design We reviewed 11 fine needle aspirates of surgically confirmed salivary gland CPA in addition to three discrepant cases in which CPA was strongly considered in the cytologic diagnosis. Results Pleomorphic adenoma was considered in the differential diagnosis, but a difinitive cytologic diagnosis was not made in the 11 cases. Acinic cell carcinoma, basal cell adenoma, adenoid cystic carcinoma; myoepithelioma and spindle cell neoplasm accounted for most of the other diagnostic considerations. The three discrepant cases included a malignant myoepithelioma, acinic cell carcinoma and mucoepidermoid carcinoma. Conclusion Familiarity with the variable aspirate appearance of CPA in addition to well-defined cytologic and architectural criteria can help establish the proper diagnosis in the majority of cases. There remain, however, few cases in which a definitive diagnosis is not possible.