Objective : It is difficult to manage patients with atypical glandular cell (AGC) cytology, as the diagnostic criteria for suspected adenocarcinoma in situ (AIS) or invasive adenocarcinoma in the presence of AGC may be unclear. We report the management of patients with AGC cytology at our hospital.

Methods : Data of a total 66 patients (mean age : 44.2 years old) referred for AGC cytology over the past 11 years were retrospectively analyzed. The results of cytology and biopsy performed under colposcopic guidance were compared with the clinical course and histological diagnosis in the patients with AGC cytology.

Results : The results of repeat cytology in the 66 patients revealed AGC in 27 cases, AIS in 3 cases, high-grade squamous intraepithelial lesion (HSIL) in 8 cases, and AGC+HSIL in the Bethesda system in 11 cases. The biopsies performed under colposcopic guidance revealed a total of 30 cases (45%) with high lesions (i.e. cervical intraepithelial lesion 2+ or AIS), which was significantly lower than the number yielded by cytology. Conization was performed in 40 patients, and histopathologic examination revealed high lesions in 36 of the 40 patients. Seven patients (17.5%) had squamous and glandular lesions. Seven patients showed positive cytology, negative biopsy and positive conization histology. Human papillomavirus (HPV) genotype was examined in 27 patients and 13 were found to be positive for high-risk genotypes.

Conclusion : Since more than half of the patients with AGC cytology showed latent malignant lesions, diagnostic conization could be considered, especially in cases positive for high-risk HPV genotypes.

Background : Secretory carcinomas are low-grade tumors that account for 10% of all salivary gland tumors.

Case : A man in his 40s presented to us with a swelling in the region anterior to his right ear that he had first noticed approximately three years ago. Magnetic resonance imaging (MRI) revealed a multilocular mass measuring approximately 4 cm in diameter. Fine-needle aspiration cytology revealed tumor cells arranged in a microfollicular papillary pattern or in flat sheets, and mucin, hemorrhage, and hemosiderin-laden foamy histiocytes in the background. The nuclei were medium-sized and eccentric, containing prominent nucleoli and slightly increased chromatin. Metachromatic secretory material was observed in the cytoplasm of some tumor cells. Histologically, the tumor, contained within a cyst, showed small circular ducts and an alveolar, papillary architecture of the tumor cells. Immunohistochemically, the tumor cells were positive for Vimentin, GATA3, S-100, Mammaglobin, and CK7 ; based on the findings, the tumor was diagnosed as a secretory carcinoma. The ETV6-NTRK3 fusion gene was detected in the tumor cells by direct sequence analysis using the Sanger method.

Conclusion : In patients with salivary gland tumors in whom cytology specimens reveal tumor cells arranged in a papillary and/or microfollicular pattern, secretory carcinoma should be considered as the most a highly likely diagnosis.

Background : Intrathyroid thymic carcinoma (ITTC) is a rare tumor. We report a case of ITTC accompanied by associated with papillary carcinoma (PC).

Case : The patient, a man in his 60s, presented with the chief complaint of dysphagia to a neighborhood hospital, where examination revealed two nodules in the left lobe of the thyroid gland. He was referred to our hospital for surgical treatment and we performed total thyroidectomy. Intraoperative frozen section diagnosis and imprint cytology were performed for the mass in the lower pole of the left lobe of the thyroid gland.

Cytologically, there were large aggregates of atypical cells with prominent nucleoli in an inflammatory background. Immunocytochemically, the tumor cells showed positive staining for CK5/6, CD5, c-kit, and p63, but negative staining for thyroglobulin, TTF-1, PAX8, and calcitonin. Histologically, the mass in the lower pole of the left lobe of the thyroid gland consisted of large polygonal cells in an insular growth pattern, showing occasional squamous differentiation, accompanied by inflammatory cell infiltration of the tumor nests. Because the immunohistochemical findings were almost the same as those of the imprint cytology, the lesion was diagnosed as an ITTC. The mass in the upper pole of the left lobe of the thyroid gland was a PC.

Conclusion : Cases of ITTC and PC occurring in the two lobes of the thyroid gland have been reported. However, there are no previous reports of the two tumors occurring in the same lobe of the thyroid gland.

Background : Gastrointestinal tract cancer is a rare cause, among solid tumors, of meningeal carcinomatosis (MC). We report two cases of gastric cancer, in which meningitis was suspected symptomatically, and the diagnosis of MC was confirmed by cerebrospinal fluid (CSF) cytology.

Cases : Case 1 was a man in his 60s who underwent gastrectomy followed by chemotherapy for advanced gastric cancer. He complained of headache and gait disturbance 7 months later, and was diagnosed with vertebral metastasis. Suspecting of meningitis, cytology of the CSF revealed atypical cells suggestive of adenocarcinoma. Case 2 was a man in his 80s who underwent gastrectomy for early gastric cancer, but was diagnosed 3 years later with another early cancer involving the remnant, which was resected endoscopically. Six months thereafter, he was hospitalized for generalized weakness, became comatose about a week after admission, and was suspected as having meningitis. Cytology of the CSF disclosed signet ring cell like atypical cells.

Conclusion : Gastric cancer rarely causes MC, but MC can occur in advanced or early stages of poorly differentiated adenocarcinoma and signet ring cell carcinoma. In gastric carcinoma patients with signs of meningeal irritation after surgery, CSF cytology is useful for early diagnosis of MC.

Background : Merkel cell carcinoma (MCC) in the oral region is very rare and has been reported to show spontaneous regression. We report a case of MCC that was detected at the lingual margin, which disappeared spontaneously from the primary site.

Case : A man in his 40s visited clinic complaining of a swelling on the right lingual margin. The tumor disappeared by 2 weeks, however, it recurred and biopsy of the recurrent tumor revealed atypical cells below the squamous epithelium. Immunohistochemistry revealed positive staining for neuroendocrine markers and the tumor was diagnosed as a neuroendocrine tumor, and resection of the right side of the tongue was performed with cervical lymph node dissection. Atypical cells were found in the lymph nodes, but no atypical cells were found in the resected tongue specimen, suggesting spontaneous regression of the primary tongue tumor. The diagnosis of MCC was made based on the findings of immunohistochemistry and electron microscopy, and the spontaneous regression. Chemotherapy and radiation therapy were administered, and no recurrence has been observed until the time of writing.

Conclusion : Since the differential diagnosis of the lymph node findings, it is necessary to make a comprehensive judgment by immunohistochemistry and electron microscopy.

Background : Sarcomatoid urothelial carcinoma arising from the urinary tract, especially the ureter, is very rare. We report the cytological and histological features of a case of sarcomatoid urothelial carcinoma of the right ureter, with a review of the literature.

Case : A 67-year-old man presented to his physician with gross hematuria. Abdominal CT revealed a nodular shadow in the right ureter. Right upper tract urinary cytology revealed many atypical cells, both dispersed singly as well as in aggregates. Right nephroureterectomy was performed, and histopathology of the resected specimen showed invasive urothelial carcinoma with a sarcomatoid component. Immunostaining of the cytologic and histologic specimens for GATA3 and p63 showed positive staining for both. Based on the findings, we made the final diagnosis of sarcomatoid urothelial carcinoma of the right ureter.

Conclusion : Sarcomatoid urothelial carcinoma is a group of urothelial carcinomas with a poor prognosis, that require rapid and accurate histopathological diagnosis. When a large number of isolated or aggregated atypical cells are found on urinary cytology, it is important to consider the possibility of sarcomatoid urothelial carcinoma and perform confirmatory immunohistochemistry for GATA3 and p63.

Background : Fibrolamellar carcinoma (FLC) is a rare variant of hepatocellular carcinoma that predominantly affects relatively young persons with no underlying liver disease.

Case : Herein, we present the case of a 37-year-old woman, who was diagnosed as having a rapidly evolving FLC. On admission, she was diagnosed as having multiple liver tumors, with multiple lymph node metastases. Tests for both hepatitis B antigens and hepatitis C antibodies were negative, and the serum α-fetoprotein level was within normal limits. A tentative diagnosis of FLC was made based on the findings of fine-needle aspiration cytology (FNAC) of material obtained from an enlarged cervical lymph node. The tumor cells were large, discohesive, and polygonal in shape, with abundant granular cytoplasm. We also noted some neoplastic cells containing “pale bodies.” On immunocytochemical analysis, the neoplastic cells showed strongly positive staining for arginase-1 and cytokeratin 7, and less pronounced staining for CD68. The patient died 20 days after admission, and an autopsy examination confirmed the diagnosis of FLC with general metastases and pulmonary lymphangitis carcinomatosa.

Conclusion : Although the diagnosis of FLC can pose challenges, recognition of the specific morphological and immunohistochemical features of FLC, even by FNAC of material obtained from a metastatic site, can facilitate diagnosis.

Background : Epithelioid schwannoma is a peculiar histological subtype of schwannoma, and its occurrence in the salivary gland is extremely rare. Herein, we report a case of epithelioid schwannoma arising from the parotid gland.

Case : A 50-year-old man presented with a 1-year history of a left parotid gland swelling. Pleomorphic adenoma and myoepithelial tumors were suspected from the findings of fine-needle aspiration cytology (FNAC) of the lesion. Therefore, a left superficial parotidectomy was performed, and imprint cytology of the operated specimen revealed tumor cells with round- to spindle-shaped nuclei containing fine-granular chromatin and small, but conspicuous nucleoli, and clear-to-granular cytoplasm, forming syncytial cell clusters. Histologically, the well-defined tumor was composed of round- to spindle-shaped cells scattered in isolation, in small nests, or in cord-like or sheet-like arrangement, within a myxomatous and fibrous stroma. No necrosis or mitoses were evident. Immunohistochemically, tumor cells showed positive staining for S-100 protein, but negative staining for epithelial and myoepithelial markers. The Ki-67 labeling index was less than 1%. Finally, the tumor was diagnosed as an epithelioid schwannoma.

Conclusion : When syncytial cell clusters consisting of bland round to spindle-shaped cells are observed in FNAC of salivary gland tumors, the possibility of epithelioid schwannoma should be borne in mind in the differential diagnosis.