A Rare Tumor of Intravascular Myopericytoma in the Right Renal Vein.

Myopericytoma, a perivascular myoid neoplasm, is commonly found in the dermis or subcutaneous tissues; however, its occurrence in visceral organs is unusual. Here, we present an extremely rare tumor of intravascular myopericytoma of the right renal vein. A 44-year-old man was incidentally diagnosed with a mass in the right kidney during a routine checkup. A nephrectomy was performed because the urologist suspected renal cancer. A gross examination of the resected specimen revealed a well-circumscribed brown mass in the renal hilum. Histologically, the tumor showed a concentric multilayered proliferation of spindle cells surrounding blood vessels. Immunohistochemical staining showed that the spindle-shaped tumor cells were negative for desmin but positive for α-smooth muscle actin and h-caldesmon, indicating their myoid nature. We confirmed that the tumor was located in the right renal vein because it was encased within a thick wall that was desmin-positive and contained elastic fibers, as shown by Elastica van Gieson staining. The patient was diagnosed with an intravascular myopericytoma of the right renal vein. There are several differential diagnoses for renal mesenchymal tumors, including angiomyolipoma. This emphasizes the importance of considering these uncommon tumors when examining nephrectomy specimens.

Transcriptional repressor GATA binding 1 (TRPS1) immunoexpression in normal skin tissues and various cutaneous tumors.

BACKGROUND: Transcriptional repressor GATA binding 1 (TRPS1) is a transcription factor recently shown to play a role in the development of breast and liver cancer. Here, we evaluate TRPS1 immunoexpression in normal skin tissues and various cutaneous tumors. METHODS: TRPS1 immunohistochemistry was performed in 109 cases of primary cutaneous tumors and 19 cases of metastatic carcinomas. TRPS1 expression was also evaluated in the normal skin tissues. RESULTS: The normal epidermis was TRPS1-. In contrast, the eccrine apparatus, epithelial compartment of the hair follicles, hair papilla, sebaceous glands, and anogenital mammary-like glands were TRPS1+. In primary cutaneous tumors, TRPS1 positivity varied in poroma (2/3), nodular hidradenoma (4/5), spiradenoma (4/4), cutaneous mixed tumor (5/5), trichilemmal cyst (7/8), proliferating trichilemmal tumor (1/3), pilomatricoma (9/9), sebaceoma (2/5), extramammary Paget disease (13/13), sebaceous carcinoma (2/2), actinic keratosis (3/10), Bowen disease (7/12), and squamous cell carcinoma (1/5) cases. All cases of seborrheic keratosis, basal cell carcinoma, Merkel cell carcinoma, and malignant melanoma were TRPS1-. All metastatic breast carcinoma cases (8/8) were highly positive for TRPS1, while all but one of the other metastatic tumor cases were TRPS1-. CONCLUSIONS: TRPS1 immunoexpression was observed in several skin appendages and cutaneous tumors.

A Unique Case of Intracranial Amelanotic Melanoma with BRAF V600E Mutation Successfully Treated via Molecular-targeted Therapy.

Melanoma carries a high risk of brain metastasis. A small subset of metastatic melanomas, known as amelanotic melanomas, does not present black coloration, reflecting a lack of melanin pigmentation. Here, we report a case of B-Raf proto-oncogene (BRAF) V600E mutation associated with a metastatic brain tumor caused by the amelanotic melanoma. A 60-year-old man was transferred to our department following acute onsets of left upper limb paralysis and convulsion. In the brain imaging, multiple lesions in the right frontal lobe and left basal ganglia were detected, and the presence of an enlarged left axillary lymph node was revealed. Consequently, we removed the right frontal lesion and performed a biopsy of the left axillary lymph node. Histological analysis of both specimens indicated an amelanotic melanoma, and genetic testing revealed a BRAF V600E mutation. The residual intracranial lesions were treated with stereotactic radiotherapy and molecular-targeted therapy, with dabrafenib and trametinib as the systemic treatment. Based on the Response Evaluation Criteria in Solid Tumors, we determined that the patient achieved complete remission (CR) under uninterrupted molecular-targeted therapy over a period of 10 months. After the temporary withdrawal of dabrafenib and trametinib to avoid hepatic dysfunction, a new intracranial lesion appeared. CR of this lesion was achieved following reinstatement of the two drugs. These results suggest that, under limited conditions, molecular-targeted therapy can produce a sustained response against the intracranial metastasis of melanoma, and the therapy with reduced dose is still effective against a recurrent case after cessation of the therapy due to the toxicity.

Concurrent lung adenocarcinoma hidden among multiple shadows of COVID-19 pneumonia: A rare and instructive case report.

A 40-year-old man was admitted with a diagnosis of COVID-19 pneumonia. Although most of multiple ground-glass opacities and consolidations on computed tomography improved, a round ground-glass opacity with consolidation remained unchanged and was suspected to be a part-solid nodule of lung adenocarcinoma. Pathologic diagnosis of resected tumor was papillary adenocarcinoma.

A case of multiple lung carcinoid tumors localized in the right lower lobe.

Typical pulmonary carcinoid (TC) tumors are low-grade neuroendocrine tumors and usually detected as indolent solitary tumors. We herein report a case of multiple pulmonary carcinoid tumors and tumorlets localized in the right lower lobe with no underlying lung disorders suggesting diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). A 28-year-old man with multiple 1-to-8-mm pulmonary nodules in the peripheral pulmonary parenchyma of the right lower lobe was referred to our hospital. The patient underwent a surgical biopsy. Pathological examination revealed multiple nodules composed of spindle cells, and immunohistochemistry revealed staining for chromogranin A, synaptophysin, and CD56, suggesting neuroendocrine tumors. He was diagnosed as having multiple TC tumors and tumorlets. Neuroendocrine cell hyperplasia (NECH) was also observed on some bronchioles. A follow-up CT scan after 6 months showed no changes in the sizes of the nodules and no new lesions. The present case was histopathologically compatible with DIPNECH but it occurs mainly in elderly women. The patient might be in an early stage of DIPNECH before progression to symptomatic DIPNECH. In conclusion, clinicians should consider the possibility of carcinoid tumors and tumorlets in cases with multiple pulmonary nodules even if they are localized in one lobe.

Prognosis Prediction Using Magnetic Resonance Spectroscopy and Oligoclonal Bands in Central Nervous System Methotrexate-associated Lymphoproliferative Disorder.

Central nervous system methotrexate-associated lymphoproliferative disorder (CNS-MTX-LPD) is rare, but its spontaneous regression has been observed in some patients after withdrawal of agents. We herein report three cases of primary CNS-MTX-LPD that received oral MTX for rheumatoid arthritis. Epstein-Barr virus and oligoclonal bands (OCBs) were positive, while proton magnetic resonance spectroscopy (1H-MRS) showed an elevated lipid peak and slightly elevated choline/N-acetylaspartate ratio in common. After MTX withdrawal, brain lesions showed spontaneous regression in all cases. Our patient's 1H-MRS findings and OCBs may reflect a non-monoclonal lymphoproliferative histology as benign-type lesions in CNS-MTX-LPD.

A Case Report on Apocrine Encapsulated Papillary Carcinoma of the Breast with Frank Invasion: A Diagnostic Quandary.

Encapsulated papillary carcinoma is a special type of breast cancer defined in the fifth edition of the World Health Organization breast tumor classification guidelines. Apocrine encapsulated papillary carcinoma is extremely rare, and only 10 cases have been described previously. We encountered a case of apocrine encapsulated papillary carcinoma with frank invasion. The patient was a 77-year-old woman with a painless mass in her right breast. Core needle biopsy revealed that the tumor cells had voluminous eosinophilic cytoplasm and enlarged nuclei with prominent nucleoli. We diagnosed this lesion as carcinoma with apocrine differentiation and suggested the possibility of an encapsulated papillary carcinoma. The patient underwent a right-sided mastectomy. Gross examination of the resected specimen revealed a multilobulated tumor. Microscopically, the tumor cells, which had voluminous eosinophilic cytoplasm and enlarged nuclei with prominent nucleoli, proliferated in papillary fashion with fibrous stalks in the cystic space. Myoepithelial cells were not observed around the cystic space. Frank invasion was also observed around the encapsulated papillary carcinoma. Immunohistochemistry analysis revealed that the tumor cells were negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 and positive for androgen receptor and gross cys-tic disease fluid protein 15. Based on these findings, we diagnosed this lesion as an apocrine encapsulated papillary carcinoma with frank invasion.

Relationship between Seasonal Changes in Food Intake and Energy Metabolism, Physical Activity, and Body Composition in Young Japanese Women.

We investigated seasonal changes in food intake, energy metabolism, and physical activity (PA) and explored their associations with body composition. In total, 28 women aged 20−23 years in the Kansai area of Japan participated in this year-long study spanning the winter, spring, and summer seasons. A dietary investigation was performed using the weight recording method, and the amount of histidine in the diet, which may be related to the regulation of energy intake, was calculated. Resting metabolic rate (RMR), body composition, and PA were measured using indirect calorimetry, bioelectrical impedance analysis, and uniaxial accelerometry, respectively. The results showed that energy intake was highest in winter, decreased significantly with increasing temperature, and decreased by 25% in summer. As the intake of histidine in the diet did not increase in summer, it did not seem to be involved in the suppression of energy intake. RMR was highest in winter and decreased significantly in summer by 20%. The amount of PA was low in winter, increased significantly in the spring, and decreased again in summer. Body weight increased in winter, with an accumulation of fat in the trunk and arms, and decreased in summer, with a reduction in the amount of fat. Greater energy intake and less PA in winter induced an increment in body weight despite the increase in RMR. There were no significant changes in lean body mass between the seasons; however, the muscle weight of the lower limbs increased significantly in spring and in summer compared with that in winter (p < 0.001). Thus, seasonal changes in food intake, energy metabolism, and PA occur, with resultant changes in the body composition under comfortable air-conditioned environments.

Mesenteric desmoid tumor after robot-assisted laparoscopic cystectomy with bladder replacement: a case report.

Desmoid tumors are a very rare disease associated with familial adenomatous polyposis, surgical trauma and hormonal factors. Surgical trauma is a critical trigger for sporadic desmoid tumors. Tumor development has been reported, and laparoscopic surgery has become more widely performed than the conventional open surgery. However, a few cases of desmoid tumors have developed after robot-assisted surgery. When desmoid tumors develop after cancer surgery, they are often difficult to distinguish from cancer recurrence. This differentiation is important for patients with bladder cancer because it helps determine the treatment plan. However, very few cases of mesenteric desmoid tumors after cystectomy for bladder cancer have been reported. Herein, we present a case of desmoid tumor that developed following robot-assisted laparoscopic cystectomy for bladder cancer. The tumor was resected via minilaparotomy with laparoscopic assistance for diagnostic treatment.

Risk factors of local recurrence following implant-based breast reconstruction in breast cancer patients.

BACKGROUND: The number of patients desiring implant-based breast reconstruction has been increasing. While local recurrence is observed in patients with breast reconstruction, only a few reports have focused on the risk factors for local recurrence and the prognosis after developing local recurrence. METHODS: We analyzed 387 patients who underwent implant-based breast reconstruction during the period from 2004 to 2017 in Hiroshima City Hospital. We retrospectively examined the risk factors for local recurrence and the outcomes of patients developing such recurrence after implant-based breast reconstruction. RESULTS: The median follow-up time was 59 months. The local recurrence rate was 3.1% (n = 12). The most common reason for detecting local recurrence was a palpable mass. Four patients with local recurrence had recurrence involving the skin just above the primary lesion and needle biopsy tract. All patients with local recurrence received surgery and systemic therapy and most patients received radiation therapy, all have remained free of new recurrence to date. Multivariate analysis showed lymphatic vessel invasion (HR, 6.63; 95% CI, 1.40-31.36; p = 0.017) and positive or < 2 mm vertical margin (HR, 9.72; 95%CI, 1.23-77.13; p = 0.047) to be associated with significantly increased risk of local recurrence. CONCLUSIONS: The risk factors for local recurrence following implant-based breast reconstruction were lymphatic vessel invasion and positive or < 2 mm vertical margin. Removal of the skin just above the primary lesion and needle biopsy tract and adjuvant radiation therapy might improve local outcomes. Patients with local recurrence following implant-based breast reconstruction appear to have good outcomes with appropriate treatment.

Temporal arteritis as an initial manifestation of eosinophilic granulomatosis with polyangiitis: a case report and a literature review.

A 79-year-old woman was admitted for suspected giant cell arteritis (GCA). She had suffered from dizziness, headache, jaw claudication and visual disturbance. Her medical history included bronchial asthma and parasinusitis. Her superficial temporal arteries were markedly enlarged with tenderness. Laboratory data showed eosinophilia (6968/µL) and a positive result of myeloperoxidase-ANCA. A histological examination of the biopsied artery revealed granulomatous inflammation consisting of lymphocytes and eosinophils with a multinucleated giant cell. Her conditions met both the criteria for GCA and eosinophilic granulomatosis with polyangiitis (EGPA). We finally considered that she had temporal arteritis as an initial manifestation of EGPA after a comprehensive literature review. To our knowledge, this is the first case in which temporal arteritis with a giant cell developed as an initial and sole manifestation of EGPA.

Clinicopathological features of breast cancer in Japanese female patients with Lynch syndrome.

BACKGROUND: Lynch syndrome (LS) is a predominantly inherited syndrome caused by a pathological germline mutation in one of the mismatch repair (MMR) genes. Whether breast cancer (BC) is one of the LS-associated tumors is controversial. The aim of this retrospective cohort study was to evaluate the clinical features of BC in Japanese patients with LS. METHODS: Of 38 mutation carriers, 4 females with BC were examined in this study. RESULTS: Two of the four patients had multiple BC. Their median age at the diagnosis of BC was 63 (range, 47-84) years. The TNM (6th revision) stages of the six BCs were as follows: stage I, 33% (2/6); stage IIA, 50% (3/6); and stage IIB, 17% (1/6). Histological examination revealed four scirrhous, one papillotubular, and one medullary carcinoma. The positive ratios for estrogen receptor (ER), progesterone receptor (PgR), and human epidermal growth receptor 2 (HER2) were 83.3% (5/6), 83.3% (5/6), and 16.7% (1/6), respectively. Two of the three specimens showed MSI-H and one showed MSS. These MSI-H BCs had tumor-infiltrating lymphocytes. Two of the three specimens showed an absence of MLH1 and PMS2 proteins on immunohistochemistry. The cumulative risks for a person with LS to develop BC were 4.35% at the age of 50 years, 8.70% at 60 years, and 21.5% at 70 years. CONCLUSIONS: Our study results showed BC in Japanese females with LS to be an MSI-H tumor, which was ER and PgR positive and HER2 negative.

Synchronous coexistence of liver metastases from cecal leiomyosarcoma and rectal adenocarcinoma: A case report.

Multiple liver tumors represent a challenging condition for abdominal surgeons both in the selection of technique and the rarity of diagnosis. There are no case reports on co-existence of liver metastases from both intestinal leiomyosarcoma and adenocarcinoma. The patient described in this report successfully underwent resection of both primary lesions and liver metastases in combination with chemotherapy. As for the leiomyosarcoma, the primary cecal lesion was revealed more than three years after the patient's first visit. Peritoneal, lymph-node, and lung recurrences were observed afterward, and thus surgeries on those regions were performed. Pathologically, the peritoneal and lung recurrences comprised leiomyosarcoma and the lymph-node recurrence was diagnosed as adenocarcinoma. Despite newly discovered multiple lung recurrences and regional lymph-node metastases, the patient lived a normal life for 73 mo after the initial operation based on multidisciplinary therapy. He ultimately died of liver failure due to invasive lymph-node recurrence from the rectal adenocarcinoma, in addition to multiple lung recurrences from the leiomyosarcoma. Hepatic recurrence did not occur in this patient's case, which appears to be one reason for his long-term survival.

Granulocyte Colony-Stimulating Factor-Producing Gallbladder Cancer.

We report a case of a granulocyte colony-stimulating factor (G-CSF)-producing gallbladder tumor associated with fever in a middle-aged female. Preoperative blood analysis showed leukocytosis with elevated levels of C-reactive protein and G-CSF. We resected the liver at S4a＋S5, with regional lymph node dissection and partial resection of the duodenum. Histology revealed undifferentiated carcinoma with spindle and giant cells and papillary adenocarcinoma. Immunohistochemistry revealed Stage IIIB G-CSF-producing gallbladder cancer. Postoperatively, leukocyte and serum G-CSF levels decreased to within normal limits. Adjuvant gemcitabine chemotherapy was administered for 16 months, and she has been recurrence-free for 48 months.

Low-grade B-cell lymphoma presenting primarily in the bone marrow.

Cases of low-grade B-cell lymphoma presenting primarily in the bone marrow are rare, and its clinicopathology remains unclear. We retrospectively examined patients with low-grade B-cell lymphoma presenting primarily in the bone marrow. Fourteen patients met the inclusion criteria, including 5 with lymphoplasmacytic lymphoma (LPL), 3 with chronic lymphocytic leukemia/small lymphocytic lymphoma, 2 with follicular lymphoma (FL), and 4 with low-grade B-cell lymphoma not otherwise specified (LGBCL-NOS). The median age was 69.5 years (range, 42-89 years), and a slight male predominance was noted (9 men and 5 women, 1.8: 1). Immunohistochemically, all cases were positive for CD20. One case was positive for CD138. Both cases of FL were positive for CD10 and B-cell lymphoma 2 (BCL-2), and immunoglobulin heavy locus (IgH)/B-cell lymphoma 2 rearrangement was observed by fluorescence in situ hybridization. The myeloid differentiation primary response gene (88) leucine to proline mutation was observed in 3 of 5 LPL, 1 of 2 FL, and 2 of 4 LGBCL-NOS patients. Paraproteinemia was observed in 10 patients; IgM and IgG paraproteinemia were observed in 6 and 3 patients, respectively. In this patient series, 3 patients had died at a median follow-up of 36.5 months; the cause of death of 1 LPL patient was malignant lymphoma itself. Thus, low-grade B-cell lymphoma presenting primarily in the bone marrow has various subtypes, and approximately one-third of the patients had LGBCL-NOS. The immunophenotypic features and myeloid differentiation primary response gene (88) leucine to proline mutation data of LGBCL-NOS suggested that some cases present with characteristics similar to those of LPL or marginal zone lymphoma.

[Tumor-infiltrating lymphocytes can be used to screen for lynch syndrome - a case report].

Lynch syndrome is an inherited syndrome associated with the development of colorectal and various other cancers. A 65- year-old male underwent a laparoscopic-assisted right hemi-colectomy for ascending colon cancer (cStage II). Histologically, his tumor was diagnosed as a poorly differentiated adenocarcinoma. Lymphocytic reactions, such as tumor-infiltrating lymphocytes (TIL), and Crohn's-like reactions, were observed. Genetic testing revealed the presence of a pathogenic mutation in the MLH1. In the Lynch syndrome, the most frequently observed findings include the accumulation of mutations, and an early onset of familial colon cancer. Although the case presented here did not show the typical clinical findings of Lynch syndrome, histological examination of the lymphocytic reactions proved useful for screening for Lynch syndrome. Herein, we establish the important role of the pathologist in alerting the clinician to the possibility of Lynch syndrome when the findings of TIL and Crohn's-like reactions are detected.

[A case of gastric cancer developed after pylorus-preserving gastrectomy in a patient with lynch syndrome].

Lynch syndrome is an inherited autosomal dominant disorder caused by germ-line mutation of mismatch repair genes, in which a malignant tumor develops at a young age in the colon, endometrium, stomach, or other tissues. A 54-year-old patient with gastric cancer received pylorus-preserving gastrectomy, and a genetic examination confirmed a pathological variation of the MLH1 gene. Five years after surgery, an upper gastrointestinal endoscopy revealed a residual 0 -IIa+IIc gastric tumor approximately 2 cm in size extending from the anastomotic site to the lesser curvature side of the stomach. The remaining stomach was completely removed. The final diagnosis was T1b (SM) N1M0, StageIB gastric cancer. Microsatellite instability was positive, and we attributed the cancer to Lynch syndrome. In Lynch syndrome, the risk of multicentric gastric cancer is higher than normal, and for the initial therapy, preventive total gastrectomy should be considered as an option.

[A case of HER2-positive esophagogastric junction cancer treated by using a neoadjuvant chemotherapy regimen consisting of trastuzumab].

The case herein pertains to a 78-year-old man, who was referred to our department and in whom esophagogastric junctional cancer, triggering weight loss, was indicated. A 0.75 xx circumferential Type 3 lesion was observed in the esophagogastric junction via upper gastrointestinal endoscopy, and a biopsy revealed a moderately differentiated adenocarcinoma with overexpression of human epidermal growth factor type 2 (HER2). Contrast-enhanced computed tomography (CT) showed multiple enlarged lymph nodes on the lesser curvature of the stomach. The preoperative diagnosis was T4a (SE) N2M0, Stage IIIB esophagogastric adenocarcinoma. Four courses of preoperative chemotherapy consisting of capecitabine plus cisplatin and trastuzumab were administered. The primary lesion showed cicatrization after chemotherapy, and enlarged lymph nodes were not visible on a CT scan. Total gastrectomy, splenectomy, and D2 lymph node dissection were performed. Only a small amount (approximately 5 mm) of the primary lesion remained beneath the abdominal and esophageal mucosa, and the treatment effectiveness was Grade 2. The final diagnosis was T1b (SM) N0M0, Stage IA esophagogastric adenocarcinoma. Survival with no recurrence was confirmed 6 months after surgery. These findings suggest that inclusion of trastuzumab in preoperative chemotherapy regimens improves the degree of curability of HER2-positive esophagogastric junction cancers.

[A case of lynch syndrome treated laparoscopically for multiple colon cancers].

Lynch syndrome is an inherited syndrome associated with the development of colorectal, endometrial, stomach, and other cancers; it is caused by defects in the mismatch repair genes. Such patients are at risk of developing multiple abdominal cancers after colectomy, and the presence of adhesions may render future abdominal surgeries difficult. We recommend that patients with Lynch syndrome should be considered good candidates for laparoscopic surgery. A 43-year-old Japanese man was admitted following a positive fecal occult blood test result. The patient was diagnosed with multiple colon cancers in the right colon. He had undergone endoscopic mucosal resection for a colon polyp when he was 24 years of age. Two people among his father's second-degree relatives had colorectal cancer, and he fulfilled the revised Bethesda guidelines. He underwent laparoscopic-assisted right hemicolectomy and D3 lymph node dissection. Microsatellite instability testing indicated the presence of MSI-H, and genetic testing demonstrated a pathogenic mutation of MLH-1.