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Abstract Background Perivascular epithelioid cell neoplasm (PEComa) in a child is very rare. We herein report the first malignant case of PEComa developing in the liver of a pediatric patient. Case presentation A 10-year-old boy visited a private clinic with prolonged fever of unknown etiology. Abdominal ultrasonography was performed to evaluate the fever’s origin, revealing a large tumor in the liver. He was thus referred to a nearby hospital to investigate the tumor further. Enhanced computed tomography (CT) showed a 6.8 × 5.9 × 10.5-cm solid lesion on S4 and S5. On magnetic resonance imaging (MRI), the tumor had a low signal intensity on T1 imaging and high signal intensity on T2 imaging, with partial diffusion restriction. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed a marked uptake in the mass lesion with no evidence of metastasis. The patient was negative for all tumor markers, including AFP, CEA and PIVKA-II. The results of a needle biopsy suggested hepatocellular carcinoma. The tumor’s rapid growth suggested malignancy. Hepatic segmentectomy (S4 + S5 + S8) was performed. The tumor was resected en bloc with a margin. Microscopically, the tumor showed atypical spindle, polygonal or oval-shaped cells with a high nuclear grade, and vascular invasion. Immunohistochemistry was positive for alpha-smooth muscle antigen (α-SMA), human melanin black-45 (HMB-45) and melan A. The pathological diagnosis was malignant PEComa. In the 6 months after surgery, the patient complained of shoulder pain. MRI showed a dumbbell-shaped tumor at the 2nd thoracic vertebrae, which was confirmed to be bone metastasis of PEComa. After chemotherapy, including ifosfamide and doxorubicin, vertebrectomy was performed. Two years later, thoracoabdominal CT showed a 10-cm solid mass occupying the pelvis and a 15-mm nodule in the middle lobe of the right lung. Under a diagnosis of peritoneal and lung metastases, they were surgically removed and metastasis of PEComa was pathologically confirmed. Four months after the 2nd relapse, pelvic metastasis appeared again and mTOR (mammalian target of rapamycin) inhibitor was initiated. To our knowledge, this is the first report of malignant hepatic PEComa in a pediatric patient. Conclusion Although extremely rare, malignant hepatic PEComa can develop in a child.

Abstract Background Anaplastic large cell lymphoma (ALCL) is a CD30-positive T-cell lymphoma, which is a rare type of non-Hodgkin lymphoma. ALCL rarely presents in the gastrointestinal tract, and the esophageal involvement in of ALCL is extremely rare. Case presentation An 11-year-old boy who complained of abdominal pain and cough was diagnosed with ALK-positive ALCL on the basis of systemic lymphadenopathy findings and immunohistochemistry results of pleural effusion. Although remission was observed after chemotherapy at 5 months after diagnosis, dysphagia persisted, and esophagoscopy revealed a severe stricture in the middle thoracic esophagus. At 9 months after diagnosis, allogeneic bone marrow transplantation was performed to ensure that complete remission was maintained; however, dysphagia and saliva retention did not improve. Approximately 10 months after diagnosis, esophagoscopy revealed a blind end in the middle thoracic esophagus, similar to that in congenital esophageal atresia. Subsequently, we performed minimally invasive subtotal esophagectomy under thoracoscopy and laparoscopy and gastric conduit reconstruction via the retrosternal route more than 2 years after allogeneic bone marrow transplantation. The final pathological diagnosis was esophageal atresia with esophagitis, with no malignancy. During postoperative evaluation, the patient required swallowing training for a few months, although no major complications were noted. Oral intake was possible, and complete remission was maintained at 14 month post-surgery. Conclusions Oncologists must consider the possibility of acquired esophageal cicatricial atresia as a complication during chemotherapy for ALCL. If esophageal obstruction or esophageal atresia occur and if remission is maintained, esophagectomy and esophageal reconstruction are useful treatment options for maintaining oral intake.

Abstract Background Cholangiolocellular carcinoma (CoCC) is an extremely rare disease comprising less than 1% of all primary malignant liver tumors. No effective treatment other than resection has been established. Herein, we report a case of locally advanced CoCC diagnosed as unresectable, which was successfully treated with curative resection after downsizing chemotherapy. Case presentation A 59-year-old Japanese woman with chronic hepatitis B was diagnosed with locally advanced intrahepatic cholangiocellular carcinoma. As it was difficult to perform R0 resection in the local hospital, chemotherapy combined with gemcitabine plus cisplatin was administered every 3 weeks. After a total of 10 courses of chemotherapy over 10 months the tumor was shown to be reduced in size by computed tomography imaging, and she was referred to our department for surgical resection. The effect of chemotherapy was classified as a “partial response” in the response evaluation criteria of solid tumors. After adding one course of chemotherapy, an extended left hepatectomy with resection of the caudate lobe was performed. R0 resection was achieved. Based on the pathological findings, the final diagnosis of CoCC was determined and eight courses of S-1 adjuvant chemotherapy were administered. At 14 months after the operation, the patient was alive without tumor recurrence. Conclusions Downsizing chemotherapy with gemcitabine and cisplatin may be an effective treatment strategy in locally advanced CoCC. Further evidence is required to establish an optimal strategy for the treatment of locally advanced CoCC.

Abstract Background Mesenteric cysts have various histological forms, including mesenteric cystadenomas and borderline cystic neoplasms. Primary cystadenocarcinoma of the mesentery is extremely rare; therefore, the clinical and radiological features of this tumor have not been fully elucidated. Case presentation A 50-year-old Japanese woman had a complaint of a left-sided abdominal distention. Enhanced computed tomography and magnetic resonance imaging revealed a unilocular cystic lesion measuring approximately 10 cm located in the left side of the abdomen. 18F-fluorodeoxyglucose (FDG)-positron emission tomography/computed tomography (PET/CT) revealed mottled mild FDG uptake in the cyst wall and intense FDG uptake in several mural nodules. The cystic mass with the descending colon was completely removed. Pathological examination of the specimens revealed various histologic patterns of adenocarcinoma, including mucin production in the mural nodules. We eventually diagnosed a primary cystadenocarcinoma arising from the mesentery of the descending colon. Conclusions Malignancy should be suspected in mesenteric or retroperitoneal cystic tumors with high FDG uptake, and complete resection should be performed with adequate margins.

Abstract Background When diagnosing patients with bilateral breast cancer, it is challenging to determine the relationship between multiple breast cancer lesions at the individual patient level with certainty. Case presentation A 35-year-old Japanese woman was diagnosed with a left breast cancer. She was previously diagnosed with right pT3N3M0 stage IIIC breast cancer and underwent chemotherapy with targeted therapy, radiotherapy, and endocrine therapy as adjuvant treatment after mastectomy and axillary lymph node dissection. Approximately 2 years after the first surgery, her left breast cancer was preoperatively diagnosed as a contralateral primary breast cancer, and left mastectomy and axillary lymph node dissection were performed. Histopathologically, the tumor was determined to be invasive ductal carcinoma accompanied with several intraductal components. After a second surgery, mutation analysis of her bilateral breast cancer was performed in a clinical study, which revealed that her metachronous bilateral breast tumors had the same GATA3 and CSMD1 mutations. Thus, mutation analysis strongly supported her latter left breast cancer being a metastatic lesion from the former right breast cancer. Some difficulties in diagnosing bilateral breast cancer exist when determining whether they are double primary cancers or represent contralateral breast metastasis. The existence of intraductal components is a critical piece of information for suspecting primary lesions. However, this case demonstrated that metastatic contralateral breast lesions can have intraductal components. Conclusion Herein we report a genetically proven contralateral breast metastasis with some intraductal components.

Abstract Background Synchronous double cancer of the colon and stomach accompanied by liver metastasis is rare. It is often difficult to determine an appropriate treatment strategy for multiple liver metastases of synchronous gastric cancer and colorectal cancer. Multidisciplinary treatment is required based on the progression and location of each tumor and chemotherapy for complete resection. Case presentation A 57-year-old male who complained of acute abdominal pain and fever visited his local hospital. He underwent emergent surgery for peritonitis caused by a gastric perforation. The cytodiagnosis of ascites did not show any tumor cells. There was a liver metastasis in the lateral segment of the liver. We performed a primary closure of the defect and then applied an omentum flap. After surgery, the patient was diagnosed as having synchronous cStage IV transverse colon cancer with multiple liver metastases and cStage IIB gastric cancer. The [18F]-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) showed 18F-FDG uptake by the colon tumor and multiple liver metastases, but there was no uptake in the gastric tumor or lymph nodes. We retrospectively reevaluated the CT findings from a local hospital and detected a liver nodule in segment 2/3 (from 35 to 60 mm) and segment 6 (from 26 to 57 mm), and the tumors had dramatically grown in size in only 2 months. Because complete tumor resection would be difficult, S-1 and oxaliplatin (SOX) plus bevacizumab therapy was started to control tumor progression. After 20 courses of chemotherapy, the clinical diagnosis was ycStage IV transverse colon cancer and ycStage IIa gastric cancer. We planned a two-step procedure to completely resect the primary tumors and multiple liver metastases. We first performed a laparoscopic right-colon resection+D3 lymphadenectomy and open distal gastrectomy+D2 lymphadenectomy. The patient was discharged home on postoperative day 18. After 1 month, we performed open liver resection. The pathological findings showed that the transverse colon was ypT2 (MP) with grade 2 therapeutic effects and that there were no atypical cells in the gastric tumor and multiple liver nodules (pathological complete response). Conclusion The SOX plus bevacizumab regimen could be an option for controlling tumor progression in synchronous double cancer of the colon and stomach with liver metastasis and led to the complete resection of such tumors.

Abstract Background Patients with esophagogastric junction cancer are increasing in Western and Eastern countries. Conversely, the clinical significance of surgical resection remains controversial in these patients. We report a long-term survivor of recurrent esophagogastric junction adenocarcinoma who underwent constructive multimodal therapy, including surgical resection. Case presentation A 51-year-old man underwent total gastrectomy for esophagogastric junction adenocarcinoma in 2009. In June 2010, computed tomography (CT) indicated a lung nodule and we partially resected the right lower lung. It was pathologically diagnosed as distant metastasis from esophagogastric junction cancer. After lung resection, he received adjuvant chemotherapy with S-1 for 1 year. In September 2014, CT demonstrated a swelling of the upper mediastinal lymph node with abnormal uptake on fluorine-18 fluorodeoxyglucose positron emission tomography. We performed an ultrasonography-guided needle biopsy, and he was diagnosed with lymph nodal recurrence of esophagogastric junction adenocarcinoma by pathological examination and was subsequently treated with capecitabine plus cisplatin plus trastuzumab. Since CT showed a reduction in the metastatic upper mediastinal lymph node after chemotherapy, he underwent upper mediastinal lymphadenectomy in April 2015. Following surgery, we provided radiation therapy to the upper mediastinum and chemotherapy with S-1. At the last report, the patient was alive for 8 years and 3 months since the first surgery. Conclusions This case report shows the clinical benefit of constructive multimodal therapy for recurrent esophagogastric junction adenocarcinoma.

Abstract Background Spontaneous esophageal perforation is a potentially life-threatening condition with high morbidity and mortality rates. While surgical treatment has been employed for esophageal perforation, we have adopted conservative treatment with an esophageal stent for patients in a poor physical condition because we consider controlling sepsis and improving the physical status are the highest priorities; additionally, the surgical trauma could be fatal for these patients. Case presentation A 60-year-old male complaining of left chest and back pain after vomiting was transferred to a local hospital. Computed tomography and chest X-ray examinations showed left tension pneumothorax, pneumomediastinum, and bilateral pleural effusion suspicious of spontaneous intrathoracic esophageal perforation. He was transferred to our hospital for further treatment. After arrival, he developed septic shock with acute respiratory failure. We considered that surgical treatment was too invasive and chose conservative treatment with an esophageal stent. Under general anesthesia, we first inserted a 20-Fr. trocar in the left posterior pleural space, and a large volume of the dark pleural effusion was discharged. We then performed endoscopy and found a pinhole perforation in the left posterolateral wall of the lower esophagus. We inserted both a silicon-covered esophageal stent with a check valve and a double elemental diet (W-ED) tube. We then inserted an 18-Fr. trocar into the left anterior wall. These procedures were performed less than 24 h after onset. As intensive medical care, the patient was administered broad-spectrum antibiotics and catecholamine. The two trocars and the W-ED tube were under continuous suction at − 5 cmH2O and at − 20 cmH2O every 30 s. On the 6th day, we inserted an additional thoracic drainage tube into the left pleura under CT guidance. The patient was discharged from the ICU to the general ward on the 7th day. We removed the stent almost triweekly, and the esophageal perforation was completely healed on the 45th day. He was discharged home on the 70th day. Conclusion Conservative treatment with a temporary self-expanding covered stent with a check valve, sufficient drainage, and W-ED tube nutrition was useful and effective in this unstable case of spontaneous intrathoracic esophageal perforation.

Abstract Background Primary leiomyosarcoma (LMS) of vascular origin is a rare lesion, and patients with LMS of vascular origin have poorer prognoses than patients with LMS of other origins. The inferior vena cava is the most commonly affected vessel and accounts for 60% of all vascular cases. However, LMS originating from the ovarian vein is extremely rare, and we are only aware of 15 reported cases. Therefore, we report our experience with a case of LMS originating from the right ovarian vein and review the related literature. Case presentation A 71-year-old Japanese woman with no symptoms was admitted to our hospital because of abnormal findings in a routine abdominal ultrasonography check-up. Contrast-enhanced computed tomography of the abdomen revealed a well-defined, lobulated solid mass with a diameter of 5.5 cm in the right retroperitoneal space. The mass exhibited relatively low uptake during 18F-fluorodeoxyglucose positron emission tomography. Based on these findings, the differential diagnosis included a retroperitoneal tumor, such as a desmoid tumor, leiomyoma, LMS, and malignant mesothelioma. Operative findings confirmed that the mass had originated from the right ovarian vessels, and en bloc excision was performed for the mass and the right ovarian vessels. The final pathological diagnosis was LMS originating from the right ovarian vein, and the surgical resection margins were free from tumor cells. After histological findings confirmed the LMS diagnosis, the patient underwent adjuvant radiation therapy and has not exhibited signs of local recurrence or metastasis in the 6 months after surgery. Conclusions We encountered a 71-year-old woman with LMS originating from her right ovarian vein. The prognosis of vascular LMS is generally poor. Therefore, careful follow-up will be required for our patient.

Abstract Background Perforated diverticulitis with purulent peritonitis (Hinchey III diverticulitis) has traditionally been treated with a Hartmann’s procedure in order to avoid the considerable postoperative morbidity and mortality associated with one-stage resection and primary anastomosis. Although there have been reports regarding laparoscopic lavage as the initial treatment of perforated Hinchey III diverticulitis, a formal treatment strategy has not been established yet. We performed a three-stage surgery, including laparoscopic lavage and drainage with diverting ileostomy (first stage), laparoscopic sigmoidectomy (second stage), and ileostomy closure (third stage) in a morbidly obese patient with Hinchey III diverticulitis. Case presentation A 31-year-old man who presented with abdominal pain was diagnosed with perforated diverticulitis and sent to our hospital for evaluation. He had morbid obesity (body mass index (BMI) 50 kg/m2), acute renal failure, and uncontrolled diabetes. We performed an emergency operation including laparoscopic lavage and drainage with a diverting ileostomy for this case of Hinchey III diverticulitis. Fifteen months after the first-stage surgery, we performed laparoscopic sigmoidectomy as the second stage. Finally, 5 months later, we performed ileostomy closure. The patient recovered without significant complications. Conclusion Three-stage surgery including early laparoscopic lavage and proximal diversion for morbidly obese, comorbid patients with Hinchey III diverticulitis may be indicated in the acute phase to avoid perioperative complications and permanent colostomy creation.

Abstract Background The incidence of radiation-associated angiosarcoma (RAA) of the breast has been increasing, and its prognosis is reportedly poor. It is important to remove tumor tissues completely to prevent recurrence. Case presentation We report two cases of patients with RAA of the breast. Both patients had a nodule in their remaining breast a few years after undergoing breast-conserving surgery and radiation therapy for breast cancer. The nodules were diagnosed as angiosarcoma by skin biopsy and open biopsy, respectively. To determine the extent of lesion spread, mapping biopsy was performed before surgery. Both patients underwent mastectomy, extensive skin resection, and split skin grafting. Pathological findings showed that their tumors could be completely resected. After surgery, chemotherapy was performed. Conclusion In our cases, no local or distant recurrence has been detected in either patient for over 4 years. We identified the range of tumor invasion by preoperative mapping biopsy and completely resected all tumor tissue.

Abstract Background Perforation of the horizontal duodenum is very rare due to the presence in retroperitoneal space. It depicts an unusual clinical picture and is difficult to diagnose, leading to increased morbidity and mortality. The treatment strategies are usually varied and based on small series of cases, literature reviews, and expert opinions. Case presentation Here, we presented three cases of horizontal duodenal perforation in three different clinical processes. The first case, a 30-year-old male patient, presented with abdominal pain and hematemesis after experiencing a physical assault on the previous day. Computed tomography (CT) scan showed rupture of the horizontal duodenum. It was repaired by side-to-side duodenojejunostomy. Postoperatively, he had anastomotic leakage, disseminated intravascular coagulation, and pulmonary failure and recovered after a long hospital stay. The second case, an 81-year-old female, had duodenal perforation with endoscopic coagulation of the bleeding diverticulum. Segmental resection of the duodenum and side-to-side duodenojejunostomy were performed. Postoperatively, there was slight anastomotic leakage, but surgical intervention was not needed. The third case, an 89-year-old female, was a patient with obstructive jaundice due to pancreas head carcinoma, who developed perforation of the horizontal duodenum during endoscopic retrograde cholangiopancreatography (ERCP). After unsuccessful conservative management, duodenojejunostomy at the perforated site and gastric bypass were performed. The postoperative course was uneventful. Conclusion Early suspicion and investigation is necessary for cases of abdominal injuries. CT scan is the investigation of choice. The management options should be based on the clinical condition of the patient, comorbidities, surgical expertise, existing guidelines, and available resources.

Abstract Background Malignant mesothelioma commonly arises from the pleura, but can also arise from the peritoneum, pericardium, and tunica vaginalis testis. However, malignant mesothelioma of the liver is extremely rare and coexistence with malignant mesothelioma of the greater omentum has not been described in the literature. In this case report, we present a case of multiple malignant mesothelioma of the liver and greater omentum. Case presentation A 36-year-old woman was admitted to our hospital for the evaluation of an elastic hard mass in the right upper abdomen. Abdominal contrast computed tomography showed a cystic mass measuring 13 × 14 × 11 cm in the right liver lobe with enhanced mural nodule. Abnormal accumulation was identified in the liver and lower abdominal area on 18F-fluorodeoxyglucose positron emission tomography. The patient underwent hepatectomy of the posterior segment and partial resection of the omentum. The final pathological diagnosis was low-grade multiple malignant epithelioid mesothelioma based on characteristic immunohistochemical findings. As of 6 months postoperatively, the patient has shown no disease recurrence. Conclusions We present the first case of a 36-year-old woman with multiple malignant mesothelioma of the liver and greater omentum.