Details of a Researcher - SUGITA Shintarou

Papers - SUGITA Shintarou

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ALK-positive atypical Spitz tumour with conspicuous rosette-like structures.

Tomoyuki Minowa, Tokimasa Hida, Kohei Horimoto, Junji Kato, Takafumi Kamiya, Shintaro Sugita, Masashi Idogawa, Toshiaki Saida, Tadashi Hasegawa, Takashi Tokino, Hisashi Uhara

European journal of dermatology : EJD 31 ( 2 ) 256 - 258 2021.04 [International journal]

DOI PubMed

Multiple lung lesions in a 13-year-old girl with classical nodular sclerosis Hodgkin Lymphoma.

Shuhei Adachi, Akira Takebayashi, Kotoe Iesato, Keita Igarashi, Masaki Yamamoto, Tsukasa Hori, Yukihiko Kawasaki, Taijiro Mishina, Shintaro Sugita

International journal of hematology 113 ( 3 ) 318 - 319 2021.03 [Domestic journal]

DOI PubMed

Stroke Mimics and Chameleons from the Radiological Viewpoint of Glioma Diagnosis.

Ayaka Sasagawa, Takeshi Mikami, Yusuke Kimura, Yukinori Akiyama, Shintaro Sugita, Tadashi Hasegawa, Masahiko Wanibuchi, Nobuhiro Mikuni

Neurologia medico-chirurgica 61 ( 2 ) 134 - 143 2021.02 [Domestic journal]

Gliomas are sometimes difficult to differentiate from strokes and are often misdiagnosed on magnetic resonance imaging (MRI); thus, the terms "stroke mimics" and "stroke chameleons" have been introduced. In this study, we analyzed stroke mimics and stroke chameleons in glioma and discussed the diagnostic perplexity.We retrospectively reviewed cases that were removed from lesions that were considered to be brain tumors. This study enrolled 214 patients who underwent tumor resection for suspected glioma. Clinical characteristics and radiological findings of the patients were compared between the masquerade findings group, which was further divided into two groups: the stroke chameleons and stroke mimics according to their final diagnosis, and the intelligible findings group.Stroke chameleons and stroke mimics were significantly higher in age and smaller in lesion size than the intelligible findings group. In the multivariate analysis, the predictive factor of the masquerade finding group was higher age and smaller size. Stroke mimics group has a tendency to be higher rate of hyperintensity lesion on diffusion-weighted imaging (DWI) compared with stroke chameleons group. The average period from initial diagnosis to pathological diagnosis was 13.50 days in the stroke chameleons and 61.50 days in the stroke mimics, which proved significantly different.Proper diagnosis of glioma and stroke affects a patient's prognosis, and should be diagnosed as soon as possible. However, stroke mimics and stroke chameleons caused by glioma can occur. Thus, the diagnosis of a stroke should take into consideration the possibility of a glioma in real clinical situations.

DOI PubMed

[Probable Immunoglobulin G4 (IgG4)-related Periaortitis Requiring Differentiation from Mediastinal Tumor].

Yoshiki Chiba, Wataru Arai, Miho Ohkawa, Osamu Tada, Ryunosuke Maki, Taijiro Mishina, Masahiro Miyazima, Atsushi Watanabe, Shintaro Sugita

Kyobu geka. The Japanese journal of thoracic surgery 74 ( 2 ) 108 - 111 2021.02 [Domestic journal]

A 42-year-old man with a history of suspected of Behcet's disease underwent oral steroid treatment. During follow-up, chest X-ray revealed an abnormal shadow of the mediastinum. Chest computed tomography(CT) showed a circumferential tumor around the descending thoracic aorta. Enhanced CT showed a lowly and uniformly enhanced tumor at delay phase. A mediastinal tumor was suspected, which prompted a biopsy of the periaortic tumor by video-assisted thoracic surgery (VATS). Histopathological diagnosis showed numerous immunogloblin G4 (IgG4)-positive plasma cells suggesting the possibility of IgG4-related periaortitis. However, based on the diagnostic criteria, the case was comprehensively diagnosed as probable IgG4-related periaortitis, steroid treatment may have affected blood IgG4-positive cells and tissues.

PubMed

Response to 'Is the case really a SMARCA4-deficient thoracic sarcoma?'

Kohichi Takada, Shintaro Sugita, Tadashi Hasegawa

Thoracic cancer 12 ( 1 ) 141 - 141 2021.01 [International journal]

DOI PubMed

Arteriovenous malformation in the pancreatic head initially mimicking a hypervascular mass treated with duodenum-preserving pancreatic head resection: a case report.

Takahiro Korai, Yasutoshi Kimura, Masafumi Imamura, Minoru Nagayama, Ayumi Kanazawa, Ryo Miura, Takeshi Murakami, Daisuke Kyuno, Hiroshi Yamaguchi, Kotomi Terai, Shintaro Sugita, Takayuki Nobuoka, Tadashi Hasegawa, Ichiro Takemasa

Surgical case reports 6 ( 1 ) 301 - 301 2020.12 [International journal]

BACKGROUND: The mainstay treatment for arteriovenous malformation in the pancreatic head (Ph-AVM) is standard pancreatectomy, especially pancreaticoduodenectomy (PD), or interventional endovascular treatment. We report the first case of Ph-AVM treated with duodenum-preserving pancreatic head resection (DPPHR) performed to preserve the periampullary organs. CASE PRESENTATION: A 59-year-old man presenting with back pain underwent contrast-enhanced computed tomography followed by angiography of the anterior superior pancreaticoduodenal artery. He was diagnosed with Ph-AVM and indicated for DPPHR with preservation of the periampullary organs; Ph-AVM's benign nature seldom requires lymph node dissection. During the operation, the right colon was mobilized and the omental bursa was released to expose the periampullary structures. The pancreas was transected just above the superior mesenteric vein. The inferior pancreaticoduodenal artery and papillary arteries branching from the posterior superior pancreaticoduodenal artery were carefully preserved to maintain the blood flow to the lower bile duct and papilla of Vater. The remnant pancreas was reconstructed with pancreaticogastrostomy using the modified Blumgart method. Pathological examination of the resected specimen revealed an irregular course of the arteries and veins concomitant with marked dilation throughout the pancreatic head. The patient was pathologically diagnosed with Ph-AVM. He developed hematemesis caused by a rupture of the pseudoaneurysm on postoperative day 20 and underwent coil embolization. A bilio-enteric fistula and stenosis of the common bile duct were found and treated by placement of an endoscopic biliary stent. At the 8-month follow-up, the Ph-AVM had not recurred. CONCLUSIONS: Compared to PD, DPPHR confers the clinical benefit of preserving the periampullary organs, although further studies are needed to confirm this. Therefore, the choice of this procedure should be based on the surgical morbidities and long-term outcome of the patient.

DOI PubMed

Myoepithelioma of soft tissue and bone, and myoepithelioma-like tumors of the vulvar region: Clinicopathological study of 15 cases by PLAG1 immunohistochemistry.

Keiko Segawa, Shintaro Sugita, Tomoyuki Aoyama, Tomoko Takenami, Hiroko Asanuma, Yui Kojima, Yoshiaki Inayama, Tadashi Hasegawa

Pathology international 70 ( 12 ) 965 - 974 2020.12 [International journal]

Authorship： Corresponding author

We demonstrated the clinicopathological findings of 13 myoepitheliomas of soft tissue and bone (MESTBs) and two myoepithelioma-like tumors of the vulvar region (MELTVRs), focusing on the association between nuclear atypia and clinical course, and the utility of immunohistochemistry (IHC) of pleomorphic adenoma gene 1 (PLAG1) for the pathological diagnosis of these tumors. Of the 13 MESTBs, eight, one and four cases exhibited mild, moderate and severe nuclear atypia, respectively. Two cases with venous invasion showed severe nuclear atypia and both died of advanced disease. Two MELTVR cases showed moderate nuclear atypia and had no evidence of disease after surgery. On IHC, 12 of 13 (92.3%) MESTBs showed PLAG1 immunoreactivity and none of the MELTVRs expressed PLAG1. In addition, MELTVRs showed loss of INI1 expression. In contrast, all MESTBs retained INI1 expression. Fluorescence in situ hybridization detected EWSR1, FUS and PLAG1 rearrangement in 5 (38.5%), 0 (0%) and 2 (15.4%) of the 13 MESTBs, respectively. No EWSR1, FUS and PLAG1 rearrangement were observed in the METLVRs. In conclusion, MESTBs with both severe nuclear atypia and venous invasion would be indicative of malignant potential. PLAG1 might be a useful IHC marker in MESTB diagnosis.

DOI PubMed

Case series of three patients with hereditary diffuse gastric cancer in a single family: Three case reports and review of literature.

Masahiro Hirakawa, Kohichi Takada, Masanori Sato, Chisa Fujita, Naotaka Hayasaka, Takayuki Nobuoka, Shintaro Sugita, Aki Ishikawa, Miyako Mizukami, Hiroyuki Ohnuma, Kazuyuki Murase, Koji Miyanishi, Masayoshi Kobune, Ichiro Takemasa, Tadashi Hasegawa, Akihiro Sakurai, Junji Kato

World journal of gastroenterology 26 ( 42 ) 6689 - 6697 2020.11 [International journal]

BACKGROUND: Hereditary diffuse gastric cancer (HDGC) is a familial cancer syndrome often associated with germline mutations in the CDH1 gene. However, the frequency of CDH1 mutations is low in patients with HDGC in East Asian countries. Herein, we report three cases of HDGC harboring a missense CDH1 variant, c.1679C>G, from a single Japanese family. CASE SUMMARY: A 26-year-old female (Case 1) and a 51-year-old male (father of Case 1), who had a strong family history of gastric cancer, were diagnosed with advanced diffuse gastric cancer. After genetic counselling, a 25-year-old younger brother of Case 1 underwent surveillance esophagogastroduodenoscopy that detected small signet ring cell carcinoma foci as multiple pale lesions in the gastric mucosa. Genetic analysis revealed a CDH1 c.1679C>G variant in all three patients. CONCLUSION: It is important for individuals suspected of having HDGC to be actively offered genetics evaluation. This report will contribute to an increased awareness of HDGC.

DOI PubMed

Surgically treated gastric melanoma of unknown primary: A case report from a 10-year survivor.

Toshiyuki Takahashi, Shintaro Sugita, Hidetoshi Kagaya, Takayuki Morita

Pathology international 70 ( 10 ) 786 - 792 2020.10 [International journal]

We describe an extremely rare case of simultaneous double melanoma of the stomach with no other obvious primary source. The patient has survived for more than 12 years post-complete gastrectomy. The patient was a woman in her seventies who was referred for anemia by another clinic. Esophagogastroscopy revealed an ulcerated gastric tumor that was diagnosed as a gastrointestinal stromal tumor (GIST) by endoscopic biopsy. She was admitted to our hospital for further examination and treatment. Gastroscopy at our institution revealed two submucosal tumors in the gastric wall. Since no metastatic lesions were detected after systemic exploration, multiple GIST of the stomach was diagnosed, and a total gastrectomy was performed. Malignant melanoma was diagnosed in both lesions by a histopathological study of the resected stomach. The patient's postoperative progress was good, and thorough examination of the skin did not result in the discovery of any systemic neoplastic lesions which could be regarded as the source for the primary tumor. No anticancer treatments were used. The patient has survived, with no recurrence for over ten years postsurgery. Strong evidence is presented in this case for the diagnosis and treatment of gastric malignant melanoma.

DOI PubMed

Osteofibrous dysplasia-like adamantinoma treated via intercalary segmental resection with partial cortex preservation using pedicled vascularized fibula graft: a case report.

Yuji Yamamura, Makoto Emori, Nobuyuki Takahashi, Mitsumasa Chiba, Junya Shimizu, Yasutaka Murahashi, Shintaro Sugita, Kousuke Iba, Tadashi Hasegawa, Toshihiko Yamashita

World journal of surgical oncology 18 ( 1 ) 203 - 203 2020.08 [International journal]

BACKGROUND: Morphologically, osteofibrous dysplasia-like adamantinoma is thought to be intermediate between osteofibrous dysplasia and adamantinoma. Its treatment is not well established owing to its rarity. CASE PRESENTATION: We report about of a 10-year-old girl with osteofibrous dysplasia-like adamantinoma initially diagnosed as osteofibrous dysplasia and treated via intercalary segmental resection with partial cortex preservation using a pedicled vascularized fibula graft for reconstruction. Bone union was observed 9 weeks after surgery. Twenty-two months after the definitive surgery, no recurrence was observed. CONCLUSION: This case illustrates the upgrade from osteofibrous dysplasia to osteofibrous dysplasia-like adamantinoma. The surgical method may aid the treatment of osteofibrous dysplasia-like adamantinoma with incomplete cortex involvement of the tumor.

DOI PubMed

Mucinous carcinoma originating in the peritoneum diagnosed by an ascites cell block: a case report.

Sachiko Nagao, Motoki Matsuura, Masato Tamate, Shintaro Sugita, Tsuyoshi Saito

Journal of medical case reports 14 ( 1 ) 99 - 99 2020.07 [International journal]

BACKGROUND: Peritoneal carcinoma is a rare disease that is diagnosed and treated in a manner similar to ovarian cancer. In most cases, the histological type is serous carcinoma, and chemotherapy is effective. However, there are a few case reports of mucinous peritoneal carcinoma. We inferred the histological type before surgery using an ascites cell block sample, which was useful for determining the treatment plan. CASE PRESENTATION: Our patient was a 60-year-old Japanese woman. She presented with a feeling of fullness in the abdomen. A computed tomographic scan showed a large quantity of ascitic fluid and thickening of the greater omentum, as well as thickening of the peritoneum at the pouch of Douglas and diaphragm. Hence, peritoneal carcinoma was suspected. The tumor markers carcinoembryonic antigen, cancer antigen 19-9, and cancer antigen 125 were all increased, and no malignant findings were observed in the uterus or ovaries. Cells suggestive of carcinoma were found in the ascitic fluid, and immunostaining by the cell block method suggested the possibility of mucinous carcinoma. The preoperative chemotherapy strategy was changed to short courses, and tumor reduction surgery was planned. Similar to the suspicion before surgery, the pathology results indicated mucinous carcinoma, and the therapeutic effect of chemotherapy was grade 0. CONCLUSIONS: Determining whether peritoneal carcinoma is serous carcinoma is important for therapy and prognostic prediction. In this case, we encountered a patient for whom surgery was chosen because of drug therapy resistance inferred through histological type estimation using the cell block method. Inferring the histological type by cell block preparation is useful for diagnosis and treatment selection.

DOI PubMed

Detection of VHL deletion by fluorescence in situ hybridization in extraneuraxial hemangioblastoma of soft tissue.

Keiko Segawa, Shintaro Sugita, Tomoyuki Aoyama, Seiichi Minami, Kazuo Nagashima, Masumi Tsuda, Shinya Tanaka, Tadashi Hasegawa

Pathology international 70 ( 7 ) 473 - 475 2020.07 [International journal]

Authorship： Corresponding author

DOI PubMed

Genetic analyses of mosaic neurofibromatosis type 1 with giant café-au-lait macule, plexiform neurofibroma and multiple melanocytic nevi.

Tokimasa Hida, Masashi Idogawa, Masae Okura, Shintaro Sugita, Taro Sugawara, Yasushi Sasaki, Takashi Tokino, Toshiharu Yamashita, Hisashi Uhara

The Journal of dermatology 47 ( 6 ) 658 - 662 2020.06 [International journal]

Neurofibromatosis type 1 (NF1) is a genodermatosis caused by heterozygous germ line variations in the NF1 gene. A second-hit NF1 aberration results in the formation of café-au-lait macules, cutaneous neurofibroma and plexiform neurofibroma (PNF). Mosaic NF1 (mNF1), caused by a postzygotic NF1 mutation, is characterized by localized or generalized NF1-related manifestations. Although NF1 and mNF1 are associated with pigmentary skin lesions, clinically recognizable melanocytic nevi that developed over PNF have not been reported. Here, we report the first case of multiple melanocytic nevi that developed on a giant café-au-lait macule and PNF. The PNF had biallelic NF1 deletions, a whole deletion of NF1 and a novel intragenic deletion involving exons 25-30. The deletions were not detected in the blood, which resulted in the diagnosis of mNF1. Furthermore, the nevus cells had not only biallelic NF1 deletions but also NRAS Q61R, a common mutation found in congenital melanocytic nevi. These analyses revealed the coexistence of the two different mosaic diseases, mNF1 and congenital melanocytic nevi. For a diagnosis of cases with atypical NF1-like symptoms, genetic analyses using blood and lesional tissues are useful and aid in genetic counseling.

DOI PubMed

Pulmonary metastasectomy is associated with prolonged survival among patients with bone and soft tissue sarcoma.

Junya Shimizu, Makoto Emori, Yasutaka Murahashi, Tomoko Sonoda, Taijiro Mishina, Masahiro Miyajima, Atsushi Watanabe, Shintaro Sugita, Kohichi Takada, Kazuyuki Murase, Tadashi Hasegawa, Toshihiko Yamashita

Molecular and clinical oncology 12 ( 5 ) 429 - 434 2020.05 [International journal]

The aim of the present study was to evaluate the survival impact of surgical resection among patients with pulmonary metastases from bone and soft tissue sarcomas. A total of 34 consecutive patients with ≤5 pulmonary metastases from bone and soft tissue sarcomas were retrospectively reviewed. The patients included 19 men and 15 women, with a median age of 64.0 years and a median follow-up of 14.5 months. The oncological outcome was compared between patients who underwent surgical and non-surgical treatment. A total of 22 patients underwent surgery and 12 patients did not undergo surgery. The surgery group had 3- and 5 year overall survival rates of 62 and 53%, respectively. None of the patients in the non-surgery group survived to 3 years. Compared with the non-surgery group, surgery achieved significantly better 3- and 5 year overall survival rates. Pulmonary metastasectomy was associated with significantly improved survival among patients who were aged <64 years (P=0.0155), as well as those who were aged ≥64 years (P=0.0444), which indicated that age was not associated with a difference in survival between the two groups. Therefore, pulmonary metastasectomy may improve the prognosis of patients with pulmonary metastases from bone and soft tissue sarcomas.

DOI PubMed

Molecular Aberrations Associated with Seizure Control in Diffuse Astrocytic and Oligodendroglial Tumors.

Hime Suzuki, Nobuhiro Mikuni, Shintaro Sugita, Tomoyuki Aoyama, Rintaro Yokoyama, Yuto Suzuki, Rei Enatsu, Yukinori Akiyama, Takeshi Mikami, Masahiko Wanibuchi, Tadashi Hasegawa

Neurologia medico-chirurgica 60 ( 3 ) 147 - 155 2020.03 [Domestic journal]

Diffuse astrocytic and oligodendroglial tumors are frequently associated with symptomatic epilepsy, and predictive seizure control is important for the improvement of patient quality of life. To elucidate the factors related to drug resistance of brain tumor-associated epilepsy from a pathological perspective. From January 2012 to October 2017, 36 patients diagnosed with diffuse astrocytic or oligodendroglial tumors were included. Assessment for seizure control was performed according to the Engel classification of seizures. Patient clinical, radiological, and pathological data were stratified based on the following 16 variables: age, sex, location of tumor, existence of the preoperative seizure, extent of resection, administration of temozolomide, radiation therapy, recurrence, Karnofsky performance scale, isocitrate dehydrogenase 1, 1p/19q co-deletion, Olig2, platelet-derived growth factor receptor alpha, p53, ATRX, and Ki67. These factors were compared between the well-controlled group and drug-resistant seizure group. Twenty-seven patients experienced seizures; of these, 14 cases were well-controlled, and 13 cases were drug-resistant. Neither clinical nor radiological characteristics were significantly different between these two groups, though p53 immunodetection levels were significantly higher, and the frequency of 1p/19q co-deletion was significantly lower in the group with drug-resistant seizures than in the well-controlled group. In the multivariate analysis, only one item was selected according to stepwise methods, and a significant difference was observed for p53 (OR, 21.600; 95% CI, 2.135-218.579; P = 0.009). Upregulation of p53 may be a molecular mechanism underlying drug resistant epilepsy associated with diffuse astrocytic and oligodendroglial tumors.

DOI PubMed

High Specificity of BCL11B and GLG1 for EWSR1-FLI1 and EWSR1-ERG Positive Ewing Sarcoma.

Martin F Orth, Tilman L B Hölting, Marlene Dallmayer, Fabienne S Wehweck, Tanja Paul, Julian Musa, Michaela C Baldauf, Didier Surdez, Olivier Delattre, Maximilian M L Knott, Laura Romero-Pérez, Merve Kasan, Florencia Cidre-Aranaz, Julia S Gerke, Shunya Ohmura, Jing Li, Aruna Marchetto, Anton G Henssen, Özlem Özen, Shintaro Sugita, Tadashi Hasegawa, Takayuki Kanaseki, Stefanie Bertram, Uta Dirksen, Wolfgang Hartmann, Thomas Kirchner, Thomas G P Grünewald

Cancers 12 ( 3 ) 2020.03 [International journal]

Ewing sarcoma (EwS) is an aggressive cancer displaying an undifferentiated small-round-cell histomorphology that can be easily confused with a broad spectrum of differential diagnoses. Using comparative transcriptomics and immunohistochemistry (IHC), we previously identified BCL11B and GLG1 as potential specific auxiliary IHC markers for EWSR1-FLI1-positive EwS. Herein, we aimed at validating the specificity of both markers in a far larger and independent cohort of EwS (including EWSR1-ERG-positive cases) and differential diagnoses. Furthermore, we evaluated their intra-tumoral expression heterogeneity. Thus, we stained tissue microarrays from 133 molecularly confirmed EwS cases and 320 samples from morphological mimics, as well as a series of patient-derived xenograft (PDX) models for BCL11B, GLG1, and CD99, and systematically assessed the immunoreactivity and optimal cut-offs for each marker. These analyses demonstrated that high BCL11B and/or GLG1 immunoreactivity in CD99-positive cases had a specificity of 97.5% and an accuracy of 87.4% for diagnosing EwS solely by IHC, and that the markers were expressed by EWSR1-ERG-positive EwS. Only little intra-tumoral heterogeneity in immunoreactivity was observed for differential diagnoses. These results indicate that BCL11B and GLG1 may help as specific auxiliary IHC markers in diagnosing EwS in conjunction with CD99, especially if confirmatory molecular diagnostics are not available.

DOI PubMed

Pediatric angiomyxofibromatous tumor of the falx cerebri with locally destructive growth into the cranial bone.

Kentaro Tsuji, Takeo Nakaya, Kentaro Ashizawa, Fumihiro Arai, Noriyoshi Fukushima, Akira Gomi, Shintaro Sugita, Tadashi Hasegawa, Akira Tanaka

Pathology international 70 ( 2 ) 123 - 125 2020.02 [International journal]

DOI PubMed

Successful Treatment by Surgery and Lenvatinib of a Patient with Adrenal Metastasis of Papillary Thyroid Cancer.

Hajime Nakamura, Kohichi Takada, Kazuyuki Murase, Hiroki Sakamoto, Naotaka Hayasaka, Kazuma Ishikawa, Yuki Ikeda, Makoto Yoshida, Satoshi Iyama, Ko Kobayashi, Tetsuya Shindo, Shintaro Sugita, Koji Miyanishi, Masayoshi Kobune, Naoya Masumori, Junji Kato

Case reports in oncological medicine 2020 2107430 - 2107430 2020 [International journal]

Papillary thyroid cancer (PTC) is considered an indolent cancer, but some PTC patients do present with distant metastases and treatment strategies for such patients are not well established. Recently, lenvatinib, an inhibitor of multiple tyrosine kinases, has been introduced to treat patients with advanced PTC but carries a risk of serious adverse events such as hemorrhage. Here, we report a PTC patient with a left adrenal metastasis and lenvatinib-induced hemorrhage who underwent successful surgical resection and was subsequently treated with a lower dose of lenvatinib. The patient has now been in a stable state with no adverse events for nearly two years. This case highlights the importance of surgical resection of metastatic PTC and subsequent lenvatinib therapy, even when the tumor is at an advanced stage.

DOI PubMed

Exceptionally rapid response to pembrolizumab in a SMARCA4-deficient thoracic sarcoma overexpressing PD-L1: A case report.

Kohichi Takada, Shintaro Sugita, Kazuyuki Murase, Tomoki Kikuchi, Ginji Oomori, Ryo Ito, Naotaka Hayasaka, Koji Miyanishi, Satoshi Iyama, Hiroshi Ikeda, Masayoshi Kobune, Makoto Emori, Junji Kato, Tadashi Hasegawa

Thoracic cancer 10 ( 12 ) 2312 - 2315 2019.12 [International journal]

SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a new clinical entity characterized by SMARCA4 inactivation and has a dismal prognosis because of rapid growth. Effective treatments for SMARCA4-DTS have not yet been developed. Most recently, anti-programmed cell death 1 receptor (PD-1) blockade has been effective for SMARCA4-deficient lung cancer and malignant rhabdoid tumor-like tumors. Here, we describe a patient with SMARCA4-DTC who experienced a marked response to the administration of pembrolizumab. A 70-year-old female was referred to our department for treatment of SMARCA4-DTC. Positron emission tomography-computed tomography had revealed a left mediastinal tumor, peritoneal dissemination and multiple cutaneous metastases at diagnosis. Immunohistochemical analyses revealed 60% of tumor cells expressed programmed cell death ligand 1 (PD-L1). The patient was given pembrolizumab as first-line treatment. Pembrolizumab suppressed tumor growth dramatically, with only one dose leading to a partial response. Our case suggests the immunohistochemical analysis of PD-L1 expression be undertaken for patients with SMARCA4-DTS and that pembrolizumab treatment may be a promising strategy for PD-L1-positive SMARCA4-DTS.

DOI PubMed

Agminated fibroblastic connective tissue nevus in a 1-year-old boy.

Marie Ohata, Susumu Fujiwara, Ai Yoshioka, Hiroshi Nagai, Shintaro Sugita, Nozomi Yamano, Yusuke Inoue, Chikako Nishigori

Pediatric dermatology 36 ( 6 ) 997 - 998 2019.11 [International journal]

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Papers - SUGITA Shintarou