[A Case of Long-Term Survival of Anaplastic Transformation of Thyroid Cancer in Lymph Node Metastasis Due to Radiation Therapy and Lenvatinib].

An 82-year-old woman who underwent total thyroidectomy and left cervical lymph node dissection 21 years ago admitted our hospital because of left cervical pain. Neck CT scan showed a 6 cm tumor on the left clavicle. Pathological diagnosis by needle biopsy revealed poorly differentiated to undifferentiated carcinoma, positive for TTF-1, and diagnosed as thyroid cancer lymph node metastasis anaplastic transformation. Administration of lenvatinib was started after radiation therapy. Since thrombocytopenia was observed, lenvatinib was gradually reduced from 14 mg and the dose was continued at 4 mg. The tumor shrinked and the effect of chemotherapy was partial response. She survived for 3 years while continuing lenvatinib. We reported long-term survival due to radiation therapy and lenvatinib of anaplastic transformation of thyroid cancer in lymph node metastasis due to radiation therapy and lenvatinib.

[A Case of Retroperitoneal Primary Well-Differentiated Liposarcoma Treated by Laparoscopic Surgery].

A 76-year-old woman visited the hospital due to occult blood in her urine. An abdominal CT scan showed a low-density tumor inside the left iliopsoas muscle in the retroperitoneum, and a well-differentiated liposarcoma was suspected. Therefore, laparoscopic tumor resection was performed. The tumor was on the inside of the left iliopsoas muscle, without any invasion around it. The macroscopic appearance of the resected tumor showed a yellow, fat-like, solid mass and it was histopathologically diagnosed as a well-differentiated liposarcoma. We conclude that a retroperitoneal primary well-differentiated liposarcoma can be treated by laparoscopic surgery, as in our case.

[Granulomatosis with Polyangiitis Complicated with Gastrointestinal Perforation: A Case Report and Review of Literature].

  A 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes. Gastrointestinal perforation in GPA is a rare complication, and we examined the clinical features, treatment contents, and prognosis of GPA with gastrointestinal perforation from this case and previous reports. Lung involvements were complicated in all reported cases. Gastrointestinal perforations in GPA were frequent in the small intestine, occurred just before and immediately after the start of treatment, and were severe involvement with poor prognosis because of the high mortality rate (46.7%). The frequency of ear, nose and upper respiratory tract lesions in the surviving group was significantly higher than in the dead group (survival 87.5%, death 28.3%, P = 0.041). IVCY were more frequently used in the surviving group (62.5%) than the death group (16.7%), but it was not significantly. GPA complicated with gastrointestinal perforation is a severe condition with poor prognosis, but there is a possibility to improve prognosis by early diagnosis and early initiation of strong treatment.

[A case of undifferentiated gastric cancer that recurred with portal vein tumor thrombus].

A 69-year-old man with a malignant tumor of the stomach underwent distal gastrectomy with partial resection of the abdominal wall, transverse colon, and lateral segment of the liver. Based on a detailed pathological examination of the resected specimen, a diagnosis of undifferentiated gastric cancer was established. Six months after the operation, during postoperative adjuvant chemotherapy with S-1, diagnostic imaging, including CT and positron emission tomography (PET), revealed a portal vein tumor thrombus and diffusely spreading metastases in the posterior segment of the liver. Despite chemotherapy with S-1/CDDP, the metastatic tumors continued to grow rapidly. The patient died 10 months after the operation. On autopsy, the portal vein tumor thrombus was observed to be composed of undifferentiated cancer cells invading into the liver parenchyma through hepatic sinusoids, with the metastatic tumors replacing 60% or more of the entire liver.

[Nonfunctioning pancreatic neuroendocrine tumor with extensive calcification].

A 54-year-old woman exhibited pancreatic calcification on abdominal ultrasonography. Diagnostic imaging revealed a 20-mm mass with a 12-mm calcification in the tail of the pancreas. The mass was weakly enhanced in the early phase of contrast-enhanced CT. We performed pancreatectomy and splenectomy. Histopathological diagnosis was a nonfunctioning pancreatic neuroendocrine tumor (PNET), grade 2. This is a rare case of PNET with extensive calcification.

Two-stage treatment with hepatectomy and carbon-ion radiotherapy for multiple hepatic epithelioid hemangioendotheliomas.

Hepatic epithelioid hemangioendothelioma (HEH) is a rare neoplasm of vascular origin with variable malignant potential. Because most patients with this condition have multiple bilobar lesions, liver transplantation is the standard treatment, and hepatectomy is much less frequently indicated. We describe a case of a 35-year-old woman with unresectable multiple bilobar HEH successfully treated by combination treatment with hepatectomy and carbon-ion radiotherapy. This case is very meaningful since it demonstrated the effectiveness of carbon-ion radiotherapy for HEH and the possibility of expanding the curative treatment options for multiple bilobar hepatic tumors.

Hemobilia associated with a giant thrombosed aneurysm of the hepatic artery requiring hepatectomy.

Hemobilia is an unusual and potentially catastrophic cause of gastrointestinal bleeding. Although hepatic artery aneurysm is a cause of hemobilia, nontraumatic cases are infrequently reported. Herein, we describe the case of a giant hepatic artery aneurysm requiring hepatectomy because of repeated hemobilia in a patient with Marfan syndrome. A 53-year-old man presented to our hospital with sudden epigastric pain and jaundice. Abdominal computed tomography showed a giant hepatic arterial aneurysm in the porta hepatis, and emergency endoscopic retrograde cholangiography revealed hemobilia. Assuming that the aneurysm caused the hemobilia, we performed an abdominal angiogram for treatment. The study revealed a thrombosed aneurysm along with tortuous abnormal vessels in the periphery of the left hepatic artery, which appeared to surround the aneurysm. Therefore, we embolized the left hepatic artery, and immediate hemostasis was achieved. Rebleeding occurred 3 times thereafter, and each time, transarterial embolization was performed, resulting in prompt but only temporary hemostasis. Then, emergency left hemihepatectomy and resection of the aneurysm were performed. Pathologic examination of the resected specimen revealed that the aneurysm was completely thrombosed and organized; however, abnormal arterioles proliferated between the aneurysmal wall and the bile duct. The unique feature of this case was that the abnormal arterioles induced by the organized hepatic artery aneurysm, not the aneurysm itself, caused the hemobilia.

[Case with gradually enlarging lymphoepithelial cyst of the pancreas that necessitated enucleation].

A 54-year-old man exhibited a pancreatic mass on abdominal ultrasonography. Diagnostic imaging with endoscopic ultrasonography, computed tomography, and magnetic resonance imaging revealed that the mass comprised various internal structures and was not connected to the pancreatic duct. Over a period of 4 years and 2 months, the mass increased from 22mm to 32mm in diameter. Laparoscopic enucleation was performed, and a histopathological diagnosis of a lymphoepithelial cyst (LEC) of the pancreas was obtained. LEC is rare and seldom reported in the literature. Although it is considered to be benign, most case reports indicate that they tend to increase in size. This indicates that LEC should be carefully monitored if surgery is not performed after diagnosis.

[A surgically resected case of AFP and PIVKA-II producing gastric cancer with hepatic metastasis].

A 78-year-old man was admitted for workup for a liver tumor. Both serum AFP and PIVKA-II levels were high (2260ng/ml and 806mAU/ml, respectively). Contrast-enhanced CT scan and MRI using Gd-EOB-DTPA demonstrated a liver tumor in segment 6 resembling the imaging patterns of hepatocellular carcinoma (HCC), while the upper gastrointestinal endoscopy revealed a type 2 gastric cancer in the gastric antrum. Although the liver metastasis of the gastric cancer was undeniable, we performed partial resection of segment 6 of the liver and distal gastrectomy under a preoperative diagnosis of double cancer. Histopathologically, gastric tumor consisted of two components, such as well differentiated adenocarcinoma and hepatoid adenocarcinoma. The histology of the liver tumor was similar to that of the hepatoid component in the stomach lesion. Immunohistochemical staining revealed both the gastric and the liver tumors to be positive for AFP and PIVKA-II, yielding a definite diagnosis of AFP and PIVKA-II producing gastric cancer with liver metastasis. Because many cases of this disease have liver metastases at presentation with confusing images with HCC, the diagnosis of liver tumors should be carefully differentiated in the gastric cancer patients with liver tumors, high serum AFP and PIVKA-II levels.

A case of synchronous bilateral lung cancers: EML4-ALK positive adenocarcinoma in the right lung and adenocarcinoma in situ (the former bronchioloalveolar carcinoma) in the left lung.

BACKGROUND: Recently it has been revealed that lung adenocarcinomas with distinct gene mutations or fusions are associated with particular histopathological entities. For example, epidermal growth factor receptor (EGFR) gene mutations are often associated with well differentiated adenocarcinoma of the lung with bronchioloalveolar pattern. On the other hand, echinoderm microtubule-associated protein-like 4 (EML4)-anaplastic lymphoma kinase (ALK) fusion gene in a subset of lung adenocarcinoma is related to mucinous cribriform histology. CASE PRESENTATION: Reported herein is a case of synchronous EML4-ALK positive lung adenocarcinoma and adenocarcinoma in situ in the bilateral lungs of a 55-year-old Japanese woman. The woman had EML4-ALK positive lung adenocarcinoma in the right lower lung while adenocarcinoma in situ in the left upper lung, which was EML4-ALK negative. CONCLUSION: To our knowledge, this is the first report of synchronous, bilateral lung adenocarcinomas composed of EML4-ALK positive and negative ones.

[A case of cystic duct metastasis from rectal cancer].

A 67-year-old man underwent laparoscopic low anterior resection and open medial segmentectomy of the liver for rectal cancer and liver metastasis. During the operation, a cystic duct mass, 1cm in diameter, was found. Therefore cholecystectomy with partial resection of the common bile duct was also performed. The rectal tumor and liver tumor were diagnosed pathologically as moderately differentiated adenocarcinoma. The cystic duct mass was mainly located in the lamina propria and its histological aspects were similar to the rectal cancer. Immunohistochemical staining revealed that rectal, hepatic, and cystic duct lesions were all negative for CK7 but were all positive for CK20. These findings confirmed the diagnosis of rectal cancer with metastases to the liver and the cystic duct. Metastatic cystic duct tumor is extremely rare and has never been reported in the Japanese literature.

[A case of accessory papilla of duodenum causing intestinal bleeding].

A 50-year-old man was admitted to our hospital with a chief complaint of melena. An emergency upper gastrointestinal endoscopic study revealed arterial bleeding from a duodenal submucosal tumor, 1.5cm in diameter and about 2cm in an oral direction from the papilla of Vater. Because it was not possible to stop the bleeding, an emergency resection of the tumor was performed. Macroscopically, the ulcerated tumor was approximately 1.5cm in diameter. Histopathologically, the tumor was determined to be located in the accessory papilla of the duodenum. We report here a rare case of bleeding from the accessory duodenal papilla and discuss the literature.

[A case of Cronkhite-Canada syndrome with multiple colon adenomas and early colon cancers].

A 60-year-old man presented with diarrhea and weight loss. Colonoscopy revealed multiple reddish polypoid lesions throughout the gastrointestinal tract and was diagnosed as Cronkhite-Canada syndrome (CCS). Prednisolone therapy caused regression of polyps. Some of them were suspected to be early colon cancers and adenomas. We endoscopically performed mucosal resection for 15 polyps after prednisolone therapy. Histological examination of one of polyps showed invasion of the submucosal layer and colon resection was performed. This case suggests that diagnosis and treatment are important in polyps of CCS.

[A case of small pancreatic cancer with intraductal progress].

A 60-year-old man was admitted to our department for further evaluation of main pancreatic duct dilatation detected on ultrasonography. Endoscopic retrograde cholangiopancreatography showed stenosis of the main pancreatic duct at the junction of the pancreatic head and body. Brush cytology revealed pancreatic ductal carcinoma. Histological examination of the resected pancreas showed a 15-mm in length intraductal growth of carcinoma in situ in the main pancreatic duct, 10mm of which showed microinvasion. There was also atypical hyperplasia at a branch pancreatic duct near the lesion. It was suspected to be an initial stage of pancreatic ductal carcinoma. Intraductal progression type and non-progression type are often suspected in a case showing progression of carcinoma in situ to pancreatic ductal carcinoma, and this case was thought to be intraductal progression.

The cytological characteristics of small cell change of dysplasia in small hepatic nodules.

Small cell change of dysplasia (SCD) is characterized as an initial step in hepatocarcinogenesis. Histopathological diagnosis is an important diagnostic procedure for nodular lesions in the liver. However, the biopsied specimen is so small that it is sometimes difficult to differentiate between regenerative nodules, dysplastic nodules, and hepatocellular carcinoma even histologically. To examine the usefulness of cytology in the differential diagnosis of hepatic nodular lesions, the cellular characteristics of SCD were evaluated using Papanicolaou staining and a micrometer. Sixty-four histologically diagnosed small nodular lesions in the liver were analyzed retrospectively. All cases were histologically classified according to the Terminology of Nodular Hepatocellular Lesions by the International Working party: hepatocellular carcinoma (HCC) (n=17); low-grade dysplastic nodule (LGDN) (n=26); high-grade dysplastic nodule (HGDN) (n=6); large regenerative nodule: (n=15). SCD was noted in all of the histological categories, and the proportion of SCD tended to be higher in W-HCC than in dysplastic nodules. Although the cellular size was the smallest in HGDN, the nuclear size was the largest in well-differentiated HCC (W-HCC). The nuclear/cytoplasmic ratio was higher in HGDN and W-HCC than in other nodular lesions. Hyperchromasia in W-HCC was obviously stronger than that in other nodules. SCD was frequently found in HGDN and W-HCC. The present study showed that detailed cytological findings of SCD are useful for differentiating HGDN from LGDN, and HGDN from W-HCC.

A case of multiple skeletal lesions of brown tumors, mimicking carcinoma metastases.

Brown tumor is not a true tumor, being an unusual reactive lesion in association with primary or secondary hyperparathyroidism. We report a 23-year-old woman, who initially presented with lower back pain caused by ureterolithiasis. The initial diagnosis of brown tumor was delayed, but later pain in her leg worsened and a sacral lesion was incidentally discovered on lumbar magnetic resonance imaging (MRI); multiple destructive bone lesions were then found radiologically. The radiological features of the multiple bone lesions, which mimicked multiple metastatic tumors, seemed to be those of the terminal stage of malignancy. However, pathological examination and abnormal laboratory data showing elevated serum calcium, alkaline phosphatase, and parathyroid hormone and low serum phosphate confirmed the diagnosis of brown tumor. Adenoma in the parathyroid gland was confirmed and surgically resected. The clinical symptoms of bone pain, and abnormal radiological findings and laboratory data were resolved 6 months after surgery. Synthetic analysis of the clinical, radiological, and laboratory findings was necessary for the definite diagnosis of brown tumor.

[A case of lymphocytic hypophysitis successfully treated with steroid pulse therapy].

Lymphocytic hypophysitis is an autoimmune disorder of the pituitary gland. We report a case of lymphocytic hypophysitis managed by high dose methylprednisolone pulse therapy and review the literature. A 61-year-old woman presented with severe headache. Magnetic resonance imaging (MRI) revealed a contrast enhancing pituitary mass. The patient underwent endonasal-transsphenoidal surgery of the pituitary lesion under the diagnosis of a non-secreting pituitary adenoma. Intraoperative histological findings suggested an inflammatory lesion and we performed subtotal resection of the mass. Histopathological examination confirmed the diagnosis of lymphocytic hypophysitis. Postoperatively, she received methylprednisolone pulse therapy and her severe headache resolved. This case report suggests that methylprednisolone pulse therapy may improve the clinical symptoms of lymphocytic hypophysitis.

Metastatic colon cancer from intrahepatic cholangiocarcinoma.

A 62-year-old man had been followed because of an elevated serum level of carcinoembryonic antigen without the detection of any cancer lesions. However, there was a sudden increase in the serum level of carcinoembryonic antigen, and abdominal imagings showed a hepatic tumor with peripheral intrahepatic bile duct dilatation, and a submucosal tumor at the sigmoid colon with intact mucosa. Histopathological findings showed that the hepatic tumor had perineural invasion, suggesting an intrahepatic cholangiocarcinoma, and that the colon tumor infiltrated the submucosa, while its mucosa was intact. Both tumors showed similar pathological features and were positive for cytokeratin 20 and 7. These findings suggested intrahepatic cholangiocarcinoma with metastatic sigmoid colon cancer.