Macrocystic and non-necrotic salivary duct carcinoma of the submandibular gland: A case report.

Salivary duct carcinoma (SDC) is a major malignant salivary gland tumor that usually forms a solid tumor. Non-necrotic macrocystic SDCs have rarely been reported among salivary gland tumors. A 78-year-old Japanese man with a submandibular gland tumor was evaluated radiologically, pathologically, and immunohistochemically. A multilocular lesion with a maximum size of 6 cm was radiologically observed in the left submandibular region. It had been noticed 20 years earlier. Malignant cytological result was obtained, and surgical resection was performed. Pathological examination revealed a non-necrotic, macrocystic submandibular gland tumor lined with glandular, cribriform, or papillary forms of atypical cuboidal cells. Frankly invasive components were observed in intercystic areas. Intraductal, mucoepidermoid, and secretory carcinomas were identified as pathological differential diagnoses because of their macrocystic morphology. We diagnosed SDC because there was no intraductal growth based on the lack of myoepithelial markers, diffuse immunoreactivity to gross cystic disease fluid protein15, androgen receptor, and mammaglobin and immunonegativity to S100 and p63. Postoperative positron emission tomography revealed the absence of lymph node and distant metastases. The patient was disease-free 9 months after surgery. Salivary duct carcinoma can be included in the differential diagnoses of cystic salivary gland tumors.

Current Knowledge and Prospects for Renal Hemangioblastoma and Renal Cell Carcinoma with Hemangioblastoma-like Features.

Tumors exhibiting histopathological findings similar to those of hemangioblastoma of the central nervous system (CNS-HB) rarely develop in the kidneys. Currently, renal hemangioblastoma (RHB) is considered analogous to CNS-HB; however, they differ in gross appearance, as well as immunohistochemical and molecular findings. In contrast, some renal cell carcinomas reportedly comprise distinct, clear cell renal cell carcinoma (CCRCC)- and hemangioblastoma (HB)-like areas. Initially, renal cell carcinomas with HB-like features (RCC-HBs) were considered a morphological variant of CCRCC owing to their diverse histological findings. However, the immunohistochemical and molecular findings of RCC-HBs suggest that RCC-HB is distinct from CCRCC. Additionally, one of the RCC-HBs had a focal leiomyomatous stroma and TSC2 variant, suggesting that RCC-HB and RCC with fibromyomatous stroma (RCC-FMS) might belong to the same disease entity. Therefore, we comprehensively reviewed the clinical, pathological, and molecular features of RHB, RCC-HB, and the related tumors and discussed the similarities, differences, and relationships between them. We believe that our review would serve as a foundation for further investigation on elucidating the relationship between CNS-HB, RHB, RCC-HB, and RCC-FMS.

Metastatic recurrence of lung adenocarcinoma in thyroid clinically masquerading as primary thyroid cancer and immunohistochemically positive for thyroglobulin.

We report a case of a man in his 60s with metastatic lung adenocarcinoma in the thyroid and cervical lymph nodes. The lung cancer was resected 5 years prior to presentation. The metastasis mimicked primary thyroid cancer by clinical examination and CT. However, fine-needle aspiration cytology of the thyroid and the lymph node lesions favoured lung cancer metastasis rather than thyroid cancer. Left thyroid lobectomy and lymphadenectomy were performed. Pathology confirmed the presence of an adenocarcinoma in the thyroid and two lymph nodes, which was similar to the previous lung cancer. The tumour cells in the thyroid were immunohistochemically positive for TTF1 and thyroglobulin and negative for PAX8. This is the second reported case of metastatic lung cancer in the thyroid that was focally positive for thyroglobulin. This can be a pitfall in differentiating between primary thyroid tumour and metastatic lung adenocarcinoma by pathological and cytological examination.

Schwannoma-like uterine leiomyoma with fever of unknown origin and surgical management in a middle-aged woman: A case report.

Herein, we describe a 42-year-old woman with multiple uterine leiomyomas with interesting clinical and histologic findings. She had no medical history, except for uterine myomas, which were diagnosed in her early 30s. She presented with fever and lower abdominal pain, and her symptoms did not respond to antibiotics and antipyretics. The clinical evaluation suggested that degeneration of the largest myoma might be the cause of her symptoms, and pyomyoma was suspected. As she had sustained lower abdominal pain, hysterectomy and bilateral salpingectomy were performed. Histopathological examination confirmed the presence of usual-type uterine leiomyomas without suppurative inflammation. The largest tumor showed a rare morphology with a predominant schwannoma-like growth pattern and infarct-type necrosis. Thus, schwannoma-like leiomyoma was diagnosed. This rare tumor might be one of the manifestations of hereditary leiomyomatosis and renal cell cancer syndrome; however, this patient was unlikely to have that rare syndrome. Herein, the clinical, radiological, and pathologic findings of a schwannoma-like leiomyoma are presented and we have raised the question of whether patients with schwannoma-like uterine leiomyoma are more likely to be associated with hereditary leiomyomatosis and renal cell cancer syndrome than those with usual-type uterine leiomyoma.

Kimura disease forming a human polyomavirus 6-negative parotid gland nodule with prominent squamous metaplasia in a young female: A case report.

A case of an asymptomatic 19-year-old woman with Kimura disease presenting with a nodule in the right parotid gland is presented. She had a medical history of atopic dermatitis and noticed a mass on her right-side neck. Cervical lymphadenopathy was clinically diagnosed. The initial management plan was to observe the lesion, which had enlarged from 1 cm to 2 cm in diameter 6 months later. An excisional biopsy was performed, and the pathology confirmed an eosinophil-containing inflammatory parotid gland lesion with many squamous nests and cysts, mimicking a parotid gland tumor. High serum immunoglobulin E levels, peripheral blood eosinophilia, and pathological and genetic diagnoses confirmed Kimura disease. The lesion tested negative for human polyomavirus 6. No recurrence was observed 15 months after the biopsy. The prognosis of Kimura disease without human polyomavirus 6 infection may be favorable; however, further validation of this hypothesis is required as only 5 or 6 cases of Kimura disease have been evaluated for this viral infection. Proliferative squamous metaplasia occurring in parotid gland lesions of Kimura disease is rare and may complicate the diagnostic imaging and pathological diagnosis.

Diagnostic utility of fine-needle aspiration cytology in syphilitic lymphadenitis: A case report.

Syphilis is a sexually transmitted infection that causes serious health problems without treatment. Detection of syphilis is necessary to stop the spread of the infection. Cytological screeners and pathologists may experience fine-needle aspiration cytology (FNAC) for syphilitic lymphadenitis; however, its characteristic cytological features are rarely reported. We present FNAC cytological features of syphilitic lymphadenitis in a case of a 21-year-old man. He presented with a sore throat and painful neck lymphadenopathy. His swollen and painful neck persisted even with antibiotic treatment. Necrotizing lymphadenitis and lymphoma were clinically suspected. FNAC was taken from the swollen lymph node. Cytologic findings of the specimen showed various inflammatory cells with small-sized vessels arranged in a branching/arborizing fashion. The vessels were surrounded by inflammatory cells, including plasma cells, neutrophils, and macrophages. Perivascular plasma cell cuffing was focally seen along with inconspicuous granulomas. Neutrophils appeared to involve the vascular wall. The cytological findings suggested syphilitic lymphadenitis, and clinical findings and serological tests confirmed primary syphilis with concomitant human immunodeficiency virus infection. Branching/arborizing vessels associated with many plasma cells, vascular involvement of neutrophils, and granulomas may suggest syphilitic infection if the specimen is obtained via FNAC. (189 words).

Primary adrenal Ewing's sarcoma family of tumors with tumor thrombus of the inferior vena cava: a case report.

BACKGROUND: Ewing's sarcoma is a malignant neoplasm that mainly occurs in skeletal tissue but can rarely arise in soft tissues. Recently, small round cell tumors (including Ewing's sarcoma) caused by chromosomal translocations have been collectively termed Ewing's sarcoma family of tumors. We report a rare case of primary adrenal Ewing's sarcoma family of tumors with tumor thrombus. CASE PRESENTATION: A 22-year-old Asian woman was referred to our hospital with a left retroperitoneal tumor 19 cm in diameter. Tumor thrombus was identified from the left adrenal vein to the inferior vena cava, infiltrating the right atrium. Total tumor excision with left adrenalectomy, nephrectomy, and thrombectomy was performed under hypothermic circulatory arrest, followed by seven courses of adjuvant chemotherapy. The patient has shown no signs of recurrence as of 26 months postoperatively. CONCLUSION: Radical surgery combined with systemic chemotherapy may contribute to good prognosis in patients with primary adrenal Ewing's sarcoma family of tumors.

Fibroepithelial urethral polyp with minor prostatic glands in an older man.

We describe a man in his 60s with an incidentally detected urethral polyp located in the middle section of his penile shaft. The patient had been suffering from urinary retention for 9 years due to benign prostatic hyperplasia. He was initiated on clean intermittent catheterisation to drain urine 1 year before the detection of the urethral polyp. Holmium laser nucleation of the prostate was performed, and an anterior urethral polyp (5 mm in diameter) was resected. Pathology indicated that the urethral polyp was a fibroepithelial polyp with prostate-specific antigen-immunoreactive heterotopic prostatic glands. There were no problems with urination 6 months after surgery. Fibroepithelial urethral polyps are usually congenital and are rarely described in adults. The clinicopathological features of this polyp, with its uniquely associated minor prostatic glands, are presented.

Primary syphilis presenting as a painful unilateral inguinal lymphadenopathy, without cutaneous manifestations, in a 71-year-old Japanese man: A case report.

Syphilitic infection is usually observed in young patients, and the first stage of the disease (primary syphilis) is characterized by painless cutaneous and lymph node lesions. Herein, we describe a 71-year-old Japanese man with primary syphilis that presented as unilateral inguinal lymphadenopathy without skin lesions. Originally, an incarcerated hernia was clinically suspected. The lymph node pathologically showed suppurative lymphadenitis with vague granulomas; immunohistochemistry was highly suggestive of a syphilitic infection. Primary syphilis was confirmed by positive serological tests. Syphilitic infection should be included in the clinicopathological differential diagnoses for patients with inguinal lymphadenopathy, even in elderly patients without skin lesions.

Surgical resection combined with perioperative chemotherapy for a patient with locally recurrent, previously stage IV thymic small-cell carcinoma: A case report.

An 83-year-old Japanese man visited our hospital with dyspnea and general fatigue. Computed tomography (CT) revealed a tumor in the anterior mediastinum, bilateral pleural effusion, pericardial fluid, and multiple liver nodules. We performed a CT-guided tumor biopsy, and the patient was diagnosed with thymic small-cell carcinoma, Masaoka-Koga stage classification IVb. The patient received four cycles of carboplatin and etoposide, and all lesions disappeared on CT. However, after 6 months, CT revealed a recurrent tumor in the anterior mediastinum. After one cycle of rechallenge chemotherapy, we performed extended total thymectomy followed by another three cycles of chemotherapy. More than 2.5 years after the last chemotherapy session, the patient's carcinoma did not recur. Thus, this case suggests that salvage surgery may be a treatment option for local recurrence of thymic carcinoma after complete remission with chemotherapy, even in patients with stage IV cancer.

Acute undifferentiated leukemia limited to neck lymph nodes and a large mediastinal mass.

In the 2016 update of the World Health Organization (WHO) classification of myeloid neoplasms, acute undifferentiated leukemia (AUL) was defined by a lack of lineage-specific markers. AUL has very poor prognosis and no established therapies due to its rarity. We report a case of a 31-year-old man with AUL who showed complete molecular response to an acute lymphoblastic leukemia (ALL)-based regimen and received allogeneic hematopoietic stem cell transplantation. The patient's blast cells were CD7-positive and localized to lymph nodes in the neck and to a large mediastinal mass; there was also rearrangement of the T-cell receptor delta locus. Although the tumor showed characteristics of T-cell lymphoblastic lymphoma, it was categorized as AUL based on WHO classification. This case suggests that a high-intensity conditioning regimen could be effective for rare cases of AUL that present only in the extramedullary mass, and chemotherapy for AUL should be selected based on the characteristics of the blasts.

TIMP3::ALK fusions characterize a distinctive myxoid fibroblastic tumor of the vocal cords: a report of 7 cases.

We report 7 cases of an indolent, variably myxoid tumor of the vocal cords, characterized by overt cellular atypia with large cells containing intranuclear and intracytoplasmic vacuoles, delicate curvilinear vessels, and sparse inflammatory infiltrate. Six patients were male, aged 15 to 65 years, and 1 patient was a 54-year-old female. All tumors were located in the superficial portion of the vocal cord. One patient suffered a recurrence that was completely resected; all patients with available follow-up data currently have no evidence of disease. The tumors contained alternating areas with myxoid stroma and more compacted regions with tumor cells organized in short fascicles, interwoven with delicate curvilinear vasculature. Overt cellular atypia with large cells containing intranuclear and intracytoplasmic vacuoles or resembling ganglion cells was present in all cases but mitoses and necrosis were absent. ALK immunostaining was positive in all cases, while most tumors were negative for smooth muscle actin. Targeted RNA-sequencing revealed an identical TIMP3::ALK fusion with exon 1 of TIMP3 gene being fused with exon 12 of ALK gene in all analyzable cases. For various reasons discussed, it remains unclear whether this tumor represents a mere subtype of IMT or a separate entity. Nevertheless, it is a morphologically distinct and diagnostically challenging lesion that needs to be recognized by surgical pathologists in order to prevent overdiagnosis in this clinically very delicate area.

Adrenal Schwannoma in an Elderly Man: A Case Report and Literature Review.

Schwannoma is a common mesenchymal neoplasm; however, adrenal schwannoma is rare, and it is frequently misdiagnosed as adrenal cortical adenoma. We herein report a 91-year-old Japanese man with right adrenal schwannoma that was pathologically diagnosed after adrenalectomy. To our knowledge, this is the first case of adrenal schwannoma in the oldest patient and with the longest follow-up period reported, including radiological images from 10 years earlier.

Flexible Magnifying Endoscopy with Narrow Band Imaging for Diagnosing Uterine Cervical Neoplasms: A Multicenter Prospective Study.

We aimed to investigate the diagnostic ability of magnifying endoscopy with narrow band imaging (ME-NBI) for cervical intraepithelial neoplasia grade 2 or worse (CIN2+). This was a multicenter prospective study. Eligible patients had positive Pap smear results or follow-up high-grade cytology or CIN3 diagnosed in referring hospitals. Patients underwent ME-NBI by a gastrointestinal endoscopist, followed by colposcopy by a gynecologist. One lesion with the worst finding was considered the main lesion. Punch biopsies were collected from all indicated areas and one normal area. The reference standard was the highest histological grade among all biopsy specimens. The primary endpoint was the detection rate of patients with CIN2+ in the main lesion. The secondary endpoints were diagnostic ability for CIN2+ lesions and patients' acceptability. We enrolled 88 patients. The detection rate of ME-NBI for patients with CIN2+ was 79% (95% CI: 66-88%; p = 1.000), which was comparable to that of colposcopy (79%; p = 1.000). For diagnosing CIN2+ lesions, ME-NBI showed a better sensitivity than colposcopy (87% vs. 74%, respectively; p = 0.302) but a lower specificity (50% vs. 68%, respectively; p = 0.210). Patients graded ME-NBI as having significantly less discomfort and involving less embarrassment than colposcopy. ME-NBI did not show a higher detection ability than colposcopy for patients with CIN2+, whereas it did show a better patient acceptability.

Twenty years' use of expanded polytetrafluoroethylene sheet for an artificial cardiac pacemaker.

Physicians can prolongedly use expanded polytetrafluoroethylene sheets for fixation of artificial cardiac pacemakers to avoid pacemaker lead displacement. The sheets can also be used to prevent implant rejection in patients with metal allergies.

Sarcoidosis-lymphoma syndrome with portal hypertension: diagnostic clues and approach.

Sarcoidosis-lymphoma syndrome associated with portal hypertension is very rare. A 68-year-old female presented with a 5 kg weight loss in 6 months. Soluble interleukin-2 receptor activity was increased and total platelet count was decreased. Contrast-enhanced computed tomography showed the presence of hepatosplenomegaly and a 3 cm-sized tumor in segment 3 of the liver. The hepatic venous catheterization showed mild portal hypertension. On fluorodeoxyglucose-positron emission tomography/computed tomography, progressive malignant lymphoma was suspected. However, bone marrow biopsy showed multiple noncaseating granulomas. A laparoscopic liver biopsy revealed that the liver tumor had features of Hodgkin lymphoma. There were multiple noncaseating epithelioid granulomas in the portal tracts of the liver. Splenectomy for splenomegaly and partial hepatectomy for the liver tumor were performed. Pathological examination of the resected specimens revealed multiple noncaseating epithelioid granulomas in the liver and spleen. Histopathology of the liver tumor confirmed classic Hodgkin lymphoma with mixed cellularity. We conclude that hepatic venous catheterization, positron emission tomography/computed tomography, and pathological examinations of bone marrow, liver, and spleen are crucial for the diagnosis of sarcoidosis-lymphoma syndrome associated with portal hypertension.

The Acute Onset of Autoimmune Hepatitis During Pregnancy in the Absence of Hypergammaglobulinemia and Autoantibodies.

The onset of autoimmune hepatitis (AIH) during pregnancy is rare and often poses a diagnostic challenge. A 29-year-old Japanese woman experienced epigastric pain and nausea during the third trimester of her third pregnancy. Three days after the symptom onset, an emergency Caesarean section was performed because of suspected acute fatty liver of pregnancy; however, the patient's liver dysfunction worsened afterward. Despite normal serum IgG concentration and absence of autoantibodies, biopsy-proven severe hepatitis with centrilobular zonal necrosis and good biochemical response to corticosteroids led to a diagnosis of AIH. Therefore, AIH should be included in the differential diagnosis of liver dysfunction during pregnancy.

Quality Assessment of Endoscopic Forceps Biopsy Samples under Magnifying Narrow Band Imaging for Histological Diagnosis of Cervical Intraepithelial Neoplasia: A Feasibility Study.

The current standard for diagnosing cervical intraepithelial neoplasia (CIN) is colposcopy followed by punch biopsy. We have developed flexible magnifying endoscopy with narrow band imaging (ME-NBI) for the diagnosis of CIN. Here, we investigated the feasibility of targeted endoscopic forceps biopsy (E-Bx) under guidance of ME-NBI for the diagnosis of CIN. We prospectively enrolled 32 consecutive patients with confirmed or suspected high-grade CIN undergoing cervical conization. Next to colposcopy, the same patients underwent ME-NBI just before conization. ME-NBI was performed, and 30 E-Bx samples were taken from lesions suspicious for high-grade CIN and 15 from non-suspicious mucosa. We recalled 82 punch biopsy (P-Bx) specimens taken from lesions suspicious for high-grade CIN under colposcopic examination before enrollment. The proportion of sufficient biopsy samples, which had an entire mucosal layer with subepithelial tissue, for the diagnosis of CIN was evaluated by both methods. Performance of targeted E-Bx for the final diagnosis of at least high-grade CIN was calculated. Seventeen P-Bx specimens were unavailable. The proportion of sufficient samples with E-Bx was 84%, which was similar to that with P-Bx (87%) (p = 0.672). The sensitivity, specificity, and accuracy of ME-NBI using E-Bx was 92%, 81%, and 88%, respectively. In conclusion, ME-NBI-guided E-Bx samples were feasible for histological diagnoses of CIN, and further investigation of its diagnostic accuracy is warranted.