Treatment With Ataluren for Wound Healing and Health Complications in a Patient With Junctional Epidermolysis Bullosa.

This case report describes an 11-year-old boy with widespread chronic wounds, frequent corneal erosions, dental caries, a tracheostomy, and failure to thrive.

Treatment of Acute Graft-versus-Host Disease in Liver Transplant Recipients.

Acute graft-versus-host disease (aGvHD) is a rare complication of liver transplantation associated with high morbidity and mortality. Death typically occurs due to complications related to severe infection, shock, and multiorgan failure. The clinical presentation involves dysfunction of multiple organ systems with overlapping symptoms that often results in a diagnostic delay. As there are a limited number of cases reported in the literature, there are no clear guidelines for treatment. Many different therapeutic measures have been utilized that target various immune system pathways, but steroids remain the first line of therapy. We report on two patients who developed aGvHD after liver transplantation who were treated with ruxolitinib, a novel Janus kinase 1/2 (JAK) inhibitor that has been shown to improve outcomes in steroid refractory cases of aGvHD after allogenic hematopoietic stem cell transplantation. We reviewed the literature to discuss various therapeutic options currently available for aGvHD after liver transplantation.

Sweet syndrome with perifollicular involvement because of koebnerization from facial hair plucking.

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an uncommon skin eruption characterized by fever, leukocytosis, and tender erythematous papules, nodules, and plaques. Histopathologically, SS lesions are characterized by marked superficial papillary edema with a dense neutrophilic infiltrate. SS is known to demonstrate both the Koebner phenomenon and pathergy. The majority of reported cases of these phenomena occur following significant cutaneous injury (e.g., biopsies, burns) rather than minor trauma such as pressure and friction. Here, we present the first known reported case of SS koebnerization secondary to minor grooming-related hair plucking. In addition, this is also the first reported case to our knowledge of SS with perifollicular involvement on histopathology.

Proliferating pilomatricoma in a 9-year-old girl.

Proliferating pilomatricoma is a rare, benign tumor of hair matrix origin that rarely occurs in children. We report the case of a 9-year-old girl with a rapidly growing, proliferating pilomatricoma located on the glabella. The lesion was embolized and surgically excised, with histopathological examination of the tissue confirming the diagnosis of proliferating pilomatricoma.

Primary livedoid vasculopathy associated with mononeuritis multiplex.

A 40-year-old woman presented with painful ulcerations on the bilateral lower extremities. A biopsy confirmed the diagnosis of livedoid vasculopathy (LV). She was treated initially with aspirin and pentoxifylline, and with the addition of dipyridamole she has had no recurrence of her ulcerations to date. Despite this positive response to treatment she reported numbness and paresthesias in her legs. Nerve conduction studies confirmed a diagnosis of mononeuritis multiplex. This case highlights mononeuritis multiplex as a rarely described complication of LV, and suggests that early recognition of symptoms and a multidisciplinary approach are necessary for optimal management of this condition.

[Case of Spindle Cell Carcinoma of the Breast Resistant Neoadjuvant Chemotherapy].

We report a rare case of spindle cell carcinoma of the breast which grew rapidly during neoadjuvant chemotherapy. A 72- year-old female was presenting with chief complaint of a mass in the right breast; a tumor about 20 mm in size. Core needle biopsy of tumor revealed invasive ductal carcinoma and fine needle aspiration cytology of axillary lymph node was Class Ⅴ. So she was diagnosis breast cancer as cT2N1M0, cStage ⅡB. The tumor subtype was triple negative breast cancer (TNBC). She received the neoadjuvant chemotherapy by FEC100. After FEC 4 courses, we detected a huge and rapid growing breast mass of 40 mm by CT. She was administered received mastectomy and axillary lymph node dissection after 4 months from initial contact. Pathological finding was spindle cell carcinoma of the breast. Postoperatively, she was treated with weekly PTX for a total of 12 courses and radiation therapy for a right chest wall and supraclavicular fossa. Although the tumor was resistant for neoadjuvant chemotherapy, she is alive and well without metastasis for more than 3 years.

[A Case Altered Consciousness Due to 5-Fluorouracil-Induced Hyperammonemia in a Patient with Recurrent Colorectal Cancer].

We report a case of altered consciousness related to hyperammonemia due to FOLFIRI plus bevacizumab therapy in a patient with recurrent colorectal cancer and renal dysfunction.A 76-year-old man received third-line chemotherapy for left mediastinal lymph node metastasis.He complained of diarrhea on the evening of the same day, and mental confusion on day 3 of the first FOLFIRI therapy.He had a JCS of Ⅲ(200).The laboratory results revealed a marked hyperammonemia.5 - fluorouracil(5-FU)-induced hyperammonemia was diagnosed and the patient was ventilated and managed with branchedchain amino acid solutions, lactulose, and hemodialysis in the ICU.After hemodialysis, the blood ammonia level reduced to the normal limits, and the symptoms of encephalopathy resolved on the following day.He was discharged home on the 19th day of hospitalization.5 -FU-containing therapy should be carefully administered in patients with renal dysfunction.Herein, we report a case of 5-FU-induced hyperammonemia with literature considerations.

[Four Cases of Appendiceal Mucinous Neoplasm Treated with Laparoscopic Surgery].

Appendiceal mucinous neoplasm has a risk for pseudomyxoma peritonei caused by appendiceal perforation.It has been reported that laparoscopic surgery is more risky than open surgery.We investigated 4 patients who underwent laparoscopic surgery for appendiceal mucinous neoplasm.The median age was 69.5(49-85).There were 3 males and 1 female.Three cases of partial laparoscopic resection of the cecum and 1 case of ileocecal resection with lymph node dissection were performed. The pathology was low-grade mucinous neoplasm in all cases.The median hospital stay was 6 days, and there were no postoperative complications(CD Grade 3 or higher)or hospital death.As for long-term results, peritoneal pseudomyxoma developed in 1 case, which had already ruptured at the time of surgery.There were no recurrences in other cases.This result suggests that laparoscopic surgery for appendiceal mucinous neoplasm is safe with optimal selection of the procedure and a protective technique.

[Gastrectomy for Bleeding Gastric Cancer During Ramucirumab plus Paclitaxel Therapy-A Case Report].

A 66-year-old woman diagnosed with advanced Stage Ⅳ gastric cancer(T4aN3M1[LYM])received RAM plus wPTX as third-line chemotherapy(ramucirumab 8mg/kg on the 1st and 15th day, paclitaxel 80mg/m2 on the 1st, 8th, and 15th day).After receiving 3 courses of this treatment, para-aortic lymphadenopathy had diminished but anemia had progressed because of tumor hemorrhage.Six weeks after the last administration of RAM, an open distal gastrectomy with D1 plus lymph node dissection and Billroth Ⅰ reconstruction was performed.The patient was discharged on the 9th day after surgery without complications such as postoperative bleeding and delayed wound healing.RAM plus wPTX therapy was restarted 6 weeks after the operation.Postoperative late complication there is no adverse event including adenocarcinoma and continues the same therapy at present.The perioperative treatment under the use of angiogenesis inhibitor has risk of postoperative bleeding and wound healing delay and includes surgery timing of treatment may be difficult.In our case, surgery was performed 6 weeks after the final administration of RAM.

[A Case in Which Acotiamide Hydrochloride Was Effective for Reflux Esophagitis after Distal Gastrectomy with Billroth â Reconstruction for Gastric Cancer].

In a 65-year-old woman, anemia was observed during outpatient follow-up after right lung cancer surgery, and upper gastrointestinal endoscopy was performed for examination.Gastrointestinal endoscopy revealed a Type 2 tumor on the small curvature in the middle part of the stomach, and she was diagnosed with gastric cancer.Distal gastrectomy with D2 lymph node dissection and BillrothⅠ reconstruction was performed for the gastric cancer.There were no postoperative complications, and she was discharged on the ninth day after surgery.The pathological diagnosis was gastric cancer, ML, Less, Type 2, 67×55×15 mm, muc>sig>por, pT4a(SE)N2M0, fStage ⅢB.S -1 adjuvant chemotherapy was administered, but then discontinued in the second course due to the development of adverse events.Reflux symptoms appeared after the surgery, and her dietary intake was poor.Her body weight and serum albumin level at 3 and 5 months after surgery were 51 kg and 52.5 kg, respectively, and 3.2 g/dL and 2.7 g/dL, respectively.Because there was no improvement in the reflux symptom, oral administration of acotiamide hydrochloride was initiated 7 months after the surgery.After initiating oral intake of acotiamide hydrochloride, her dietary intake improved, and her body weight and serum albumin level at 11 and 15 months after surgery were 54 kg and 57 kg, respectively, and 3.0 g/dL and 2.7 g/dL, respectively.Peritoneal recurrence was observed 23 months after surgery, and her oral intake decreased, but the recurrence of reflux symptoms was not observed.Acotiamide hydrochloride could be an option for the treatment of reflux symptoms after gastrectomy.

[A Case of Perforated Intestinal Malignant Lymphoma Induced to Remission by Multidisciplinary Therapy].

A 61-year-old man had a sudden severe abdominal pain and visited our hospital. He was diagnosed with intestinal perforation, given the peritoneal irritation symptoms, the thickening of the intestinal wall and free air as shown on enhanced abdominal computed tomography. He then underwent emergent surgery. A tumor with small perforation was found on the intestine about 15 cm distant from the terminal ileum. Partial resection of the ileum was performed. Microscopically, a type 2 tumor of 70×50mm in diameter, was observed in the resected intestine. The tumor was diagnosed as diffuse large B-cell lymphoma via immunochemical staining. He received 6courses of R-CHOP therapy after surgery without recurrence. A primary intestinal malignant lymphoma is easily perforated, which lead to poor prognosis of the patient. We report a case of perforated intestinal malignant lymphoma, which was induced to remission by multidisciplinary therapy.

[A Case of Long-Term Survival with Multidisciplinary Treatment in Unresectable Advanced Gastric Cancer Found with Ovarian Tumor].

Although chemotherapy is the standard treatment for unresectable advanced gastric cancer, its prognosis is poor and the median survival time is only around 10 months. With some literature consideration, we report that ovarian metastasis triggered the diagnosis of unresectable advanced gastric cancer with long-term survival through multidisciplinary treatment. This is the case of a 69-year-old woman, who was diagnosed with ovarian tumor and underwent right extracorporectomy and omentum resection in 2011. Pathological diagnosis suspected adenocarcinoma. In February 2012, EGD found a type 4 tumor in the upper portion of the pyloric area of the stomach. She was diagnosed with gastric cancer with Stage IV(T4aN0M1 [ovary])ovarian metastasis. Because the primary tumor was HER2 positive, XP plus HER therapy(capecitabine 1,000mg/m2 twice a day for 14 days, CDDP 80mg/m2 every 3 weeks on the first day, and trastuzumab 8 mg/kg every 3 weeks on the first day)was administered since March 2012. No metastasis was found in the imaging examination after 8 courses of chemotherapy, and we also confirmed the reduction of the primary tumor in the EGD. Based on the images, primary resection was already possible. In December 2012, after a diagnostic laparoscopy, total gastrectomy with D2 lymph node dissection was performed. Postoperative pathology was diagnosed as Stage IV(pT4aN0M1). To control postoperative micrometastasis, capecitabine therapy(1,000mg/m2 twice a day for 14 days)was administered for 12 months starting from February 2013. Then, recurrence and metastasis were not observed during follow-up. However, in January 2017, a circumstellar stenotic tumor was found in the rectum and was diagnosed as recurrence of peritoneal dissemination through images. In February 2017, artificial ostomy(in the sigmoid colon, double-mouth type)was made. Then, the patient underwent an outpatient chemotherapy, with hospital visits, and she survives.

[A Case Report of Retroperitoneal Liposarcoma Resected Six Times for 16 Years].

We report of a long-surviving patient with retroperitoneal liposarcoma that required 6 surgeries in 16 years. A 62-year-old man was diagnosed with liposarcoma, which was first excised in 2001. Thereafter, we excised recurrences in the retroperitoneum with the left kidney in February 2004. We excised recurrences with the left half of the colon in November 2007 and February 2010 and recurrences with a part of the intestine in November 2014. This time, we excised recurrences under the right abdominal rectus muscle and near the left half of the colon. The pathological diagnosis was well-differentiated liposarcoma. There is currently no evidence of recurrence 16 years after the first recurrence excision. In this case, active excision of recurrences and identification of high-grade dedifferentiated-type liposarcomas were factors of long survival.

[A Case of Liver Metastasis of Colorectal Cancer Undifferentiated from Biliary Cystadenocarcinoma].

A 74-year-old woman was found to have a hepatic mass based on CT findings. She was diagnosed as having cecum cancer, and it was difficult to distinguish whether the hepatic mass was liver metastasis or biliary cystadenocarcinoma. We proceeded with the surgery for cecum cancer, and laparoscopic ileocecal resection with D3 lymph node dissection was performed. The histopathological diagnosis was mucinous adenocarcinoma, and the pathological stage was T3N2H1P0M1a, Stage IV. After the surgery, her CEA level was elevated, and we diagnosed the hepatic mass as a liver metastasis. A CapeOX plus bevacizumab regimen was administered but was discontinued for 2 courses due to the development of adverse effects and her decision. Gd-EOB-DTPA-enhanced MRI revealed a multilocular and lobulated mass, which was a low-intensity area in T1WI and high-intensity area in T2WI, and the mass had no significant contrast effects. These images were unspecific for liver metastasis of colorectal cancer, and we performed segmental 6 hepatectomy for diagnosis and curative surgery. A histopathological diagnosis of liver metastasis of cecum cancer was made. Here, we report a case of liver metastasis of colorectal cancer that was undifferentiated from biliary cystadenocarcinoma.

[A Case Report of Laparoscopy-Assisted Distal Gastrectomy for Early Gastric Cancer with Abortive Behçet Disease].

A 32-year-old woman with a history of undergoing upper gastrointestinal surgery was administered prednisolone for abortive Behçet's disease since 2015. In March 2017, upper gastrointestinal endoscopy revealed a 0-IIc type early stage gastric carcinoma at the posterior wall of the gastric antrum. The pathological diagnosis was signet-ring cell carcinoma. This gastric carcinoma was classified as cT1aN0M0; therefore, endoscopic submucosal dissection(ESD)was performed for the expanded indication lesion in April 2017. The pathological diagnosis after ESD indicated the need for non-curative resection; thus, laparoscopy-assisted distal gastrectomy and Billroth I anastomosis were performed in July 2017. A transient anastomotic structure was admitted after this surgery, but she was discharged from the hospital lighthearted. A preoperative important aspect was the careful examination of all intestinal tracts for ulcer lesions, and an intraoperative important aspect was hand-sewn anastomosis because using a metallic stapler conferred a high risk of ulcer lesions and suture failure. We encountered a case wherein laparoscopy-assisted distal gastrectomy was performed in a patient with abortive Behçet's disease, early stage gastric carcinoma, and a history of undergoing upper gastrointestinal surgery.

[A Case of Long-Term Survival in a Patient with Advanced Gastric Cancer with Para-Aortic Lymph Node Metastasis Who Responded to Combined Modality Therapy].

A 59-year-old woman was diagnosed with advanced gastric cancer with para-aortic and Virchow's lymph node metastases (L, Less, type 2, tub1, cT3N2H0P0M1[LYM: #16, Virchow's]). Four courses of S-1(80mg/m / 2, days 1-21)and CDDP(60mg/ m2, day 8)were administered. After the chemotherapy, CT showed that the primary tumor and para-aortic and Virchow's lymph nodes had reduced in size. The clinical stage was ycT3N1H0P0M0, stage III A. The patient underwent distalgastrectomy, D2 dissection, and sampling of the para-aortic lymph nodes(#16b1lat, #16a2int). The pathological diagnosis was L, less, type 1, por1/2, pT2N2H0P0M0CY0, pStage III B. The patient was treated with S-1(80mg/m2, days 1-28)as adjuvant chemotherapy. After 3 courses had been administered(6 months after the resection), swelling of a para-aortic lymph node (#16b1int)was detected using CT. Based on the diagnosis of recurrence in the lymph node, weekly paclitaxel(80mg/m2, days 1, 8, and 15)was administered. After receiving 9 courses of weekly paclitaxel, the swelling of the lymph node disappeared, and the response evaluation was complete response. She discontinued the chemotherapy 5 years and 9 months after the surgery. To date, she has survived more than 6 years after surgery without recurrence. We report a long-surviving patient with advanced gastric cancer with para-aortic lymph node metastasis who received combined modality therapy.