A long-term surviving patient with invasive thymoma who underwent radiotherapy and/or resection for chest wall, intrathoracic and intrapelvic recurrent tumors]

A 49-year-old woman with myasthenia gravis who underwent left panpleuropneumonectomy for an invasive thymoma that disseminated through the left thoracic cavity. After six year, radiotherapy was conducted on the recurrent tumor in the left anterior chest wall. Two years later, the recurrent tumors in the intrapelvic and intrathoracic cavities were resected. It was thought that long-term survival was obtained by combining radiotherapy and surgical treatment in view of the patient's general condition, and of the recurrent invasive thymoma present in this case.     

Recurrent fibrolamellar hepatocellular carcinoma with biliary invasion: Successful resection

A jaundiced 17-year-old man was diagnosed as having a local recurrence of fibrolamellar hepatocellular carcinoma 2 years and 4 months after left hepatic trisegmentectomy with total caudate lobectomy had been performed. The patient had a tumor occupying the upper part of the extrahepatic and intrahepatic bile ducts. Complete resection of the recurrent tumor was carried out. The patient remains well 3 years after the second surgery. Fibrolamellar hepatocellular carcinoma, a rare type of liver cancer, is a well defined disease entity with distinct clinical and histopathological features and a favorable prognosis. The good prognosis seems to warrant aggressive surgical intervention in patients with recurrences. Therefore, additional surgery for tumor recurrence should be considered. To our knowledge, this is the first report of a case in which a recurrent tumor of fibrolamellar hepatocellular carcinoma invaded the entire bile duct wall was successfully resected.     

Long-term surviving patient with inferior vena cava tumor thrombus and extrahepatic metastasis after spontaneous ruptured hepatocellular carcinoma

We present a case of long-term survival in a patient with inferior vena cava tumor thrombus (IVCTT) and extrahepatic metastasis after resection for spontaneous ruptured hepatocellular carcinoma (HCC). The patient was a 73-year-old Japanese man previously diagnosed with chronic hepatitis B. He was referred to our emergency room and diagnosed with spontaneous ruptured HCC. The patient was immediately treated with transcatheter arterial embolization, and we then performed second-stage hepatic resection 50 days later. Although des-gamma-carboxy prothrombin was reduced to a normal level after hepatectomy, it gradually increased and computed tomography showed a disseminated tumor in the diaphragm near S2 of the liver with IVCTT and right atrium tumor thrombus. Recurrent HCC was treated with monthly transcatheter arterial infusion chemotherapy (TAI) and conformal radiotherapy (RT) of 40 Gy. After TAI and RT procedures, the disseminated tumor and IVCTT completely disappeared. Four years after TAI and RT procedures, the tumors were well controlled with no local recurrence. About 6−7 years after spontaneous ruptured HCC, lung metastasis and spleen metastasis were detected and resected, respectively. The patient is still alive and doing well over 7 years after spontaneous ruptured HCC.     

KL-6-producing invasive thymoma

We report a patient with KL-6-producing invasive thymoma. A 58-year-old man was admitted complaining of dyspnea and fatigability. Computed tomography of the chest revealed interstitial pneumonia and an anterior mediastinal tumor. The tumor was surgically extirpated and diagnosed as invasive thymoma. Serum KL-6 levels later increased further and another tumor was found in the liver. That liver tumor was resected and histologically diagnosed as a metastasis of thymoma. Following resection, the serum KL-6 level decreased. Tumor cells of both primary and metastatic lesions exhibited positive reactivity to immunohistochemical staining for KL-6. A review of this case is presented.     

Mesenteric arteritis.

Four patients are presented with small bowel infarction secondary to a vascular arteritis. In three patients there was a history of rheumatoid arthritis. In each patient infarcted bowel was resected and a primary anastomosis performed. In one patient the anastomosis broke down and she subsequently died. One patient died from a disseminated rectal tumour three years later. The remaining patients remain well. If operated on early, intestinal infarction due to arteritis has a good prognosis.     

Outcome of radical excision of anorectal gastrointestinal stromal tumors in Hong Kong Chinese patients.

Anorectal gastrointestinal stromal tumor (GIST) is a rare disease entity with malignant potential. Medical records of six patients (median age 68 years) with anorectal GIST who underwent surgical treatment at our institution between 1992 and 1999 were retrospectively reviewed. Four patients presented with rectal bleeding. The tumors were located in the mid and lower rectum in 4 patients and in the anal canal in 2 patients. The median tumor diameter was 4.5 cm. One patient who had undergone local excisions in another hospital presented with recurrent GIST. He refused radical excision and underwent wide local excision again. He developed recurrence 2 years later and underwent salvage pelvic exenteration, but finally died of disseminated disease. Five patients underwent initial radical excision. Among them, 3 developed recurrences (one each local, distant and both) at a median duration of 50.3 months. Two patients died of the disease, while one patient who had both local and distant recurrences resected remained alive till the end of the study period (median duration of follow-up of the 5 patients was 84.6 months). At 5 years, of 5 patients who underwent initial radical excision, 3 and 4 patients, respectively, had disease-free and overall survival. Recurrence of anorectal GIST is common despite radical excision. Nevertheless, a reasonable survival rate can be achieved.     

Recurrence of thymoma accompanied with hypogammaglobulinemia 20 years after surgery: a case report]

We reported a case of recurrence of localized thymoma accompanied with hypogammaglobulinemia (Good's syndrome) 20 years after surgery. A 74-year-old man was admitted to this hospital because of mediastinal tumor and chronic pulmonary infection. He had been thymectomised at the age of 55 because of spindle cell thymoma. After that, he had been asymptomatic until January 1997, when he began to have a recurrent productive cough, and low-grade fever. Laboratory findings revealed hypogammaglobulinemia. Percutaneous needle biopsy of the mediastinal tumor revealed spindle cell thymoma. Therefore, hypogammaglobulinemia with thymoma (Good's syndrome) accompanied with a chronic lower respiratory tract infection was diagnosed. Immunologic studies revealed a marked decrease of CD 20 positive cells and decreased lymphocyte activation under the stimuli of phytohemagglutinin and concanavalin A. The thymoma was resected in Dec 1997, but the serum immunoglobulin showed no increase at al.     

Recurrence of intrahepatic cholangiocarcinoma nine years after surgical resection

This paper reports a rare case of intrahepatic cholangiocarcinoma (ICC) which recurred nine years after surgical resection. The patient underwent right hepatic trisegmentectomy with lymph node dissection and biliary tract reconstruction for a tumor in the right lobe of the liver. The patient was diagnosed with ICC, intraductal growth (IG) type. Nine years after the operation a rise of tumor markers and a recurrent lesion in the remnant liver were recognized and the patient underwent S2 subsegmentectomy with tumor thrombectomy and biliary tract reconstruction. The resected specimen showed well differentiated adenocarcinoma, which consisted of chiefly intraductal growth component. The patient is still alive 26 months after the second operation without recurrence. It is important to observe the patient carefully cosidering that it is possible for an IG type cholangiocarcinoma to recur a long time after surgical treatment, and surgical treatment for the recurrent tumor should be tried for long survival.     

Left atrial myxoma: second recurrence 20 years after the first operation

We report about a 52-year old woman, who underwent a resection of a left atrial myxoma 20 years ago. Twelve years later she suffered from exertional dyspnea. Echocardiography revealed a recurrent myxoma. This was resected. Eight years later the woman suffered from disturbances of heart rhythm and from angina pectoris. The coronary arteries were normal, but a recurrent left atrial myxoma was found and operated. We conclude a long-term follow-up is inevitable in patients after the resection of a myxoma.     

B-cell lymphopenia and hypogammaglobulinemia in thymoma patients

Thymic tumors represent a unique neoplastic disease associated with various immune-mediated syndromes. Immune impairment is generically recognized to be associated with thymoma. Hypogammaglobulinemia and recurrent pulmonary infections in thymoma patients define Good's syndrome. Apart from sporadic reports focusing on this topic, there is still a lack of knowledge on immune assessment and clinical sequelae in thymoma patients. The present study was performed to evaluate immunoglobulin levels, CD19(+) B lymphocytes, and CD3(+) T lymphocytes in a large series of thymoma patients from a single institution. The occurrence of recurrent severe infections was related to immunological findings to identify the possible correlation with the immunodeficiency status. Eighteen patients (eight males, ten females, mean age: 56 years, range: 19-75) with a pathological diagnosis of thymic tumor were studied. Six patients suffered from clinical recurrent pulmonary infections. Blood samples were collected to measure serum immunoglobulins and analyze immunophenotype. Low T lymphocyte number was found in 22% of the patients. T lymphocytosis was present in one patient. Panhypogammaglobulinemia was found in 4 of 18 patients (22%). Conversely B lymphopenia was a frequent finding in this series of thymoma patients (9 of 18, 50%). Five of six patients (83%) with recurrent infections had B lymphopenia, while only two (33%) had panhypogammaglobulinemia. B lymphopenia often occurred in this series of thymoma patients and was related to susceptibility to recurrent infections more than hypogammaglobulinemia. Therefore, immunophenotype has to be monitored in follow-up of thymoma patients because it may reveal significant abnormalities.     

Primary adenocarcinoma arising at an ileostomy site

A case is reported of a 45-year-old man who developed a primary adenocarcinoma at the ileostomy site 23 years after proctocolectomy for ulcerative colitis. The patient underwent wide local excision of the tumor and died 13 months later with disseminated adenocarcinoma. Including the present case, a total of seven patients with ileostomy adenocarcinoma have been reported in the literature. Six patients were treated surgically for ulcerative colitis, and the other one for adenomatous polyposis coli. The diagnosis of stomal malignancy was made three to 23 years after the ileostomy (mean interval, 13 years). Of the four patients available for clinical follow-up, two died of disseminated adenocarcinoma ten and 13 months after diagnosis. Awareness of this unusual but important complication of ileostomy should lead to earlier diagnosis and treatment with improvement in prognosis.     

Strategy for pancreatic endocrine tumors

BACKGROUND/AIMS: The endocrine tumors of the pancreas are rare diseases and there is no established standard therapy for the liver metastasis of pancreatic endocrine tumors. In this study, the therapy for the pancreatic endocrine tumors was evaluated. METHODOLOGY: The endocrine pancreas tumors of 13 patients had been surgically treated. All primary tumors were completely resected. The liver metastasis was recognized in 4 patients. Partial resection of the liver was performed in 2 patients. Lipiodol-transcatheter arterial embolization was performed for synchronous unresectable liver metastases in the other 2 patients. RESULTS: The patients with no liver metastases survived without recurrence (max: 18.8 yr; mean follow-up: 9.2 yr). The patient with resected synchronous solitary liver metastasis died of recurrent multiple liver metastases 5 months after surgery. The other patient with the metachronous liver metastasis completely resected survived 13.9 years. In the 2 patients with unresectable numerous liver metastases, after lipiodol-transcatheter arterial embolization, tumor necrosis rate was more than 90% in both cases and serum gastrin level was normalized. CONCLUSIONS: Complete resection of liver metastasis is favorable, whereas lipiodol-transcatheter arterial embolization is effective for unresectable liver metastases from pancreatic endocrine tumors as palliation. Complete resection of the primary site is recommended even in the cases with unresectable numerous liver metastases.     

原发性心脏恶性肿瘤的诊断与外科治疗经验

目的总结原发性心脏恶性肿瘤的诊断和外科治疗经验。方法2003年1月至2007年12月手术治疗4例原发性心脏恶性肿瘤,其中男1例,女3例,年龄26~43岁,平均35岁,本组除1例右心房肿瘤广泛侵犯右房室环及心包仅做活检外,其余3例均在全麻低温体外循环辅助下行肿瘤切除术,根据肿瘤的部位及侵润程度,完整切除2例,姑息切除1例,同期行室间隔修补、肺动脉成形1例,二尖瓣置换1例,三尖瓣成形1例。结果全组无围术期死亡。单纯探查活检术的患者2个月后死于心力衰竭;1例右心室梭形细胞肉瘤术后辅助放、化疗,术后18个月肿瘤复发死于右心衰竭;1例左心房恶性间皮瘤手术后3个月死于双肺广泛转移;1例左心房梭形细胞肉瘤术后随访5个月无肿瘤复发。结论原发心脏恶性肿瘤预后欠佳,早期诊断,及时手术治疗辅以综合治疗可望改善预后。     

Comparison of PD-L1 immunohistochemical assays and the significance of PD-L1 expression in thymoma

Thymoma is a relatively rare malignancy, which is categorized as thymic epithelial tumor but known as the most common pathology that is developed in the anterior mediastinum. Complete resection is recommended for localized tumors and usually favorable prognosis can be obtained. However, poor survival period has been reported in unresectable cases exhibiting extensive invasion or distant metastasis, as effective chemotherapeutic regimens are restrained. We previously assessed expression of programmed death ligand 1 (PD-L1) and programmed death 1 (PD-1) and discussed their prospective application in the immunotherapy of thymic epithelial tumors. After our publication, additional studies using reliable PD-L1 antibodies, which are currently administered to predict efficacy of PD-1/PD-L1 blockade therapy were performed and further characterized PD-L1 in thymoma. Herein, recent knowledge in relation to the significance of PD-L1 expression in thymoma is reviewed based on recent findings using qualified PD-L1 clones. Most studies coherently found high expression of PD-L1 on the cell membrane and cytoplasm of tumor epithelial cells in accordance with previous reports, which is a predictive marker for effectiveness of anti-PD-1/PD-L1 drugs, even when approved PD-L1 antibodies were employed. On the other hand, PD-L1 expression on tumor infiltrating immune cells remains to be sufficiently determined. High PD-L1 expression can be expected in cases with high grade histological subtypes, such as type B2/B3 thymomas, or those with advanced stages III or IV of the disease. Interestingly, the level of PD-L1 expression was found to be upregulated after chemotherapy compared with that before, which could be explained by immunogenic cell death. The prognostic impact of PD-L1 expression in thymoma might be found only when thymic carcinoma patients were excluded. Furthermore, it also could be identified when we analyzed thymomas completely resected, distinct from biopsy and incompletely resected cases.     

Rupture of the hepatic artery after left hemihepatectomy for hilar bile duct carcinoma

Hilar bile duct carcinoma has a poor prognosis, but this has been improved in recent years by an aggressive surgical approach. We treated a 73-year-old woman who had obstructive jaundice due to bile duct carcinoma at the hepatic hilum. The jaundice decreased after percutaneous transhepatic biliary drainage. The tumor was resected with the left and caudate lobe of the liver and a part of portal vein. The right hepatic artery was located behind the common hepatic duct, and was suspected to be invaded by the tumor. We dissected the tumor from the arterial wall without carrying out combined resection of the hepatic artery. On the 6th postoperative day, the hepatic artery ruptured and the patient suffered hypovolemic shock. Resection of the hepatic artery and reconstruction were done, but the patient died 2 days later. Histological examination of the resected artery showed that the tumor had been curatively removed by dissection and that no tumor remained at the arterial wall. The rupture of the right hepatic artery was thought to have been caused by damage to the wall during the dissection procedure.     

Squamous cell carcinoma of the thymus

A 68-year-old woman was admitted with cough and dyspnea. Her chest X-ray showed right pleural effusion and a mediastinal tumor. She underwent mediastinotomy following a preoperative diagnosis of invasive thymoma. A tumor originating from the thymus had invaded the right middle lobe and pericardium, and multiple pleural dissemination was also found. Therefore, considering the patient's age and pulmonary function, we performed only subtotal resection of the tumor. The pathological diagnosis was poorly differentiated squamous cell carcinoma. The patient received irradiation and chemotherapy including Cisplatin after surgery, but she died 1 year later because of rapid progression of distant metastases.     

Recurrent carcinoma of cystic duct remnant with subcutaneous implantation in abdominal wall

A case of recurrent carcinoma of the cystic duct remnant invading the common bile duct and portal vein with subcutaneous implantation of the abdominal wall is presented. The patient was a 55-year-old woman with an abdominal wall tumor at the site of the surgical scar of a cholecystectomy, performed at a local hospital 5 years ago for symptomatic cholelithiasis. The diagnosis was made by incisional biopsy of the tumor, computed tomography, percutaneous transhepatic cholangiography, and angiography. She underwent extended right hepatic lobectomy with en bloc resection of the caudate lobe, extrahepatic bile duct, and portal vein. The abdominal wall tumor was resected concomitantly. Histological examination showed that both the recurrent carcinoma of the cystic duct remnant and the abdominal wall implantation were moderately differentiated adenocarcinoma. This recurrence probably could have been prevented if both the macroscopic and microscopic examinations of the resected specimen had been precisely carried out after the previous cholecystectomy and the primary carcinoma identified and treated at that time.     

Significance of multiple carcinoid tumors and tumorlets in surgical lung specimens: analysis of 28 patients

BACKGROUND: The clinical significance of multiple carcinoid tumorlets in surgical lung specimens has not been systematically analyzed. We reviewed our experience to determine the range of clinical circumstances associated with this finding. METHODS: We reviewed clinical records, available imaging, and pathology materials from patients evaluated at Mayo Clinic Rochester (from 1987 to 2000) with two or more carcinoid tumors or tumorlets in lung specimens. RESULTS: Twenty-eight of 294 patients with a diagnosis of carcinoid tumor or tumorlet had two or more lesions. Twenty-six patients (93%) were women; mean age was 65 years. Patients were categorized into three groups: multiple nodules (n = 17), solitary lung nodules on preoperative imaging (n = 7), and airflow limitation (n = 4). Approximately half of patients with multiple nodules had respiratory complaints; two patients had Cushing syndrome. Ten patients (58.8%) were suspected of having pulmonary metastases, including 7 patients with previously diagnosed malignancies. Intrathoracic lymph node metastases were present in three patients, none of whom had recurrent disease. One patient had a carcinoid tumor resected 8 years later. Extrathoracic metastases developed in another patient 3 years after presentation, and the patient was alive with disease 2 years later. Only one patient with airflow limitation had a syndrome resembling diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. CONCLUSIONS: Our series represents the largest compilation of multiple carcinoid tumors or tumorlets. Our analysis reveals that multiple carcinoid tumors or tumorlets occur most commonly in patients with multiple nodules resembling metastatic disease. Significant airflow limitation is rare. Long-term survival is excellent, although patients have persistent disease.     

Long-term survival after extended surgical resection of intrahepatic cholangiocarcinoma with extensive lymph node metastasis

We present a case of long-term survival in a patient that involved intrahepatic cholangiocarcinoma that metastasized to the paraaortic lymph nodes. A 62-year-old man underwent extended left hepatic lobectomy with caudate lobe resection, extrahepatic bile duct resection, portal vein resection and reconstruction, and middle hepatic vein resection and reconstruction with lymph node dissection for a liver tumor that was located in the caudate lobe. Histological examination of the resected specimen revealed moderately differentiated adenocarcinoma compatible with cholangiocarcinoma, and lymph node metastases were found in the area of the hepatoduodenal ligament and the paraaortic region. After surgical resection, recurrence was detected twice in the lymph nodes at the site of the left supraclavicular region. These recurrent tumors were completely eliminated by systemic chemotherapy with cisplatin or mitomycin C. The patient is now doing well 6 years and 5 months after surgical treatment. In this case, there was only one tumor, and the preoperative serum carbohydrate antigen 19-9 level was normal. In addition, heterozygosity was retained at the loci on chromosome 8p. These findings suggested that tumor in the present case was less aggressive, despite the nodal spread. The extensive surgical approach may have contributed to the long-term survival of this patient, while the recurrent tumor was sensitive to antitumoral agents.