A Case of Rapidly Growing Gastric Leiomyosarcoma: Observations over 18 Months

A gastric leiomyosarcoma in a 68-yr-old female was followed for 18 months by serial roentgenography. On initial x-ray, the lesion was diagnosed as a benign submucosal tumor. However, it rapidly increased in size, with doubling time subsequently calculated as 5 months. After 18 months' periodic observation, a gastrectomy was performed for a suspected malignancy. Histologic examination of the resected tumor revealed a leiomyosarcoma originating in the muscular layer of the stomach. It appears that the poor prognosis of leiomyosarcoma is related to its rapid growth; in addition, it is seldom detected at an early stage. Follow-up of the patient with a gastric submucosal tumor should be done first, 6 months after the initial examination, and thereafter, be done continuously according to the patient's condition. It is useful in differentiating a benign from a malignant myogenic tumor.     

Reevaluation of Prognostic Factors in Gastric Leiomyosarcoma

Objectives : The aim of our study was to reevaluate the prognostic factors in gastric leiomyosarcomas, particularly those under 6 cm, which, because or improved diagnostic techniques, make up a large proportion of this series, in contrast to previously puhlislied works. Methods : The clinicopathological features in 83 patients with gastric leiomyosarcoma were reviewed. Cox's proportional hazards regression analysis was used to determine the prognostic factors. Results : Sixty of 84 leiomyosar-comas (71.4%) were less than 6 cm in diameter. In university analysis, the following factors were considered to he unfavorable, with statistical significances: 1) presence of distant metastasis and/or direct invasion of adjacent structures at diagnosis (hazard ratio 36.60), 2 ) ≥4 mitoses per 20 high-power fields (25.39), 3 ) severe nuclear atypia (16.74), 4 ) presence of ulceration of overlying gastric mucosa (12.57), and 5 ) diameter ≥6 cm (5.57). Among these factors, mucosal ulceration has been identified as a new factor associated with poor prognosis. Histological. the tumors with ulceration showed more severe nuclear atypical ( p < 0.05), necrosis ( p < 0.01), and a higher incidence of mitosis ( p < 0,01), compared with tumors without ulceration. In multivariate analysis, factors 1, 2, and 3 were statistically significant, although neither size nor ulceration was significant. Conclusions : The presence of distant metastasis and/or direct invasion, high mitotic rate, and severe nuclear atypia were confirmed to he unfavorable prognostic indicators. Mucosal ulceration, indicating high proliferative activity of the tumor, even if small, suggests a poor prognosis, although it was not significant in multivariate analysis.     

Pneumonia After Endoscopic Resection for Gastric Neoplasm

Background

Pneumonia following endoscopic procedures may affect the clinical course and prolong hospital stay.

Aim

To investigate the incidence and risk factors for pneumonia after endoscopic resection (ER) for gastric neoplasm.

Methods

Subjects who underwent ER for gastric neoplasm at the Asan Medical Center from January 1997 to March 2013 were included. To investigate risk factors, control patients were randomly selected from these subjects.

Results

Of the 7,149 subjects who underwent ER for gastric neoplasm, 44 (0.62 %) developed pneumonia. The median age of these 44 patients was 68 years (range 31–82 years), and the male to female ratio was 3:1. Twenty-five of the pneumonia patients (56.8 %) were smokers, and 8 (18.2 %) had underlying pulmonary diseases. The median procedure time was 23 min (range 2–126 min), and pathologic diagnoses included adenocarcinoma (n = 29), dysplasia (n = 10), and hyperplastic polyp (n = 5). Compared with the control group, smoking (current smoker vs. never smoker, odds ratio [OR] 2.366, p = 0.021), total procedure time (OR 1.011, p = 0.048), and hemostasis time (OR 1.026, p = 0.028) were risk factors for the development of pneumonia. In multivariate analysis, age >65 years (OR 2.073, p = 0.031), smoking (current smoker vs. never smoker, OR 2.324, p = 0.023), and hemostasis time (OR 1.025, p = 0.038) were independent risk factors. All patients recovered from pneumonia, and the duration of hospital stay did not differ between patients with pneumonia and the control group (p = 0.077).

Conclusions

Whereas old age, smoking, and longer hemostasis time are risk factors for pneumonia, its incidence after ER is not associated with clinically significant adverse outcomes.     

Malignant Perivascular Epithelioid Cell Neoplasm of the Mediastinum and the Lung: One Case Report

A perivascular epithelioid cell neoplasm (PEComa) in the chest is rare, let alone in the mediastinum and lung.A 63-year-old man was admitted to our hospital with chest pain for more than 2 months and was found to have an opacity in his mediastinum and lung for 3 weeks. Enhanced chest computed tomography (CT) revealed a mass in both the left upper lobe and central anterior mediastinum. To identify the disease, a CT-guided percutaneous transthoracic needle biopsy of the upper left lung lesions was performed. The pathology result was consistent with epithelioid angiomyolipoma/PEComa. After a standard preparation for surgery, the neoplasms in the mediastinum and left lung were resected. The operative findings revealed extensive mediastinal tumor invasion in parts adjacent to the pericardium, including the mediastinal pleura, left pulmonary artery and vein, and phrenic nerve. The left lung tumor had invaded the lung membranes. The final pathologic diagnosis was malignant epithelioid angioleiomyoma in the left upper lung and mediastinum. Later, the mediastinal tumor recurred. The radiography of this case resembles left upper lobe lung cancer with mediastinal lymph node metastasis. Because this tumor lacks fat, the enhanced CT indicated that it was malignant but failed to identify it as a perivascular epithelioid cell neoplasm.This case reminds clinicians that, although most PEComa are benign, some can be malignant. As the radiology indicated, chest PEComas lack fat, which makes their preoperative diagnosis difficult. Therefore, needle biopsy is valuable for a definitive diagnosis.     

Single-center Experience in the Diagnosis and Treatment of Hepatic Perivascular Epithelioid Cell Neoplasm

Background and Aims: Perivascular epithelioid cell neo-plasms (PEComas) are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging...     

Parietal Cell Dysfunction: A Rare Cause of Gastric Neuroendocrine Neoplasm with Achlorhydria and Extreme Hypergastrinemia

A 69-year-old woman with multiple neuroendocrine neoplasms (NENs) was referred to our hospital. Although she had extreme hypergastrinemia (11,675 pg/mL), no findings that indicated types I to III gastric NENs were found. Although gastric corpus atrophy was suspected on conventional white-light imaging, findings on magnifying endoscopy with narrow-band imaging indicated no severe atrophy. A biopsy from the background fundic gland mucosa revealed no atrophic changes, parietal cells with vacuolated cytoplasm and negative findings for H⁺K⁺-ATPase. Thus, this case was diagnosed as multiple NENs with parietal cell dysfunction. Neither progression nor metastasis has been confirmed during two-year follow-up.     

胃恶性肿瘤与幽门螺杆菌L型感染的关系

应用组织切片革兰染色、免疫组化染色和电镜等技术，对136例胃恶性肿瘤进行幽门螺杆菌L型检测。结果，革兰染色幽门螺杆菌L型检出阳性率为77．2％，免疫组化染色L型检出阳性率为71．3％，两者具有一致性（P＞0．05）。此外，革兰染色在91例瘤细胞的胞浆内同时查见L型菌，透射电镜在腺癌和淋巴瘤细胞质内也见到L型菌。提示，幽门螺杆菌L型感染与胃恶性肿瘤关系密切，很可能是其致癌因子之一。     

Cardiac Epithelioid Leiomyosarcoma and the Role of Cardiac Imaging in the Differentiation of Intracardiac Masses

Primary malignant cardiac neoplasms are extremely rare and can be difficult to differentiate from other intracardiac masses. We present a case of left atrial epithelioid leiomyosarcoma and review the imaging modalities available to diagnose and characterize intracardiac masses. We demonstrate how multimodal imaging aided diagnosis and proved crucial to establishing the tumor's location, degree of invasion, and involvement of surrounding structures prior to surgical resection. Copyright © 2010 Wiley Periodicals, Inc.     

Epithelioid haemangioendothelioma: A case report

Epithelioid haemangioendothelioma of the liver is an extremely rare tumour which has only recently been recognised. This report describes the clinical, radiological and histological findings in a case of a primary epithelioid haemangioendothelioma of the liver. The case highlights some of the difficulties in establishing the diagnosis, and in its differentiation from Budd-Chiari syndrome and veno-occlusive disease. The patient has subsequently undergone successful orthotopic hepatic transplantation.     

Risk Factors for Metachronous Gastric Neoplasms in Patients Who Underwent Endoscopic Resection of a Gastric Neoplasm

Background/Aims

To identify the risk factors for metachronous gastric neoplasms in patients who underwent an endoscopic resection of a gastric neoplasm.

Methods

We prospectively collected clinicopathologic data and measured the methylation levels of HAND1, THBD, APC, and MOS in the gastric mucosa by methylation-specific real-time polymerase chain reaction in patients who underwent endoscopic resection of gastric neoplasms.

Results

A total of 257 patients with gastric neoplasms (113 low-grade dysplasias, 25 high-grade dysplasias, and 119 early gastric cancers) were enrolled. Metachronous gastric neoplasm developed in 7.4% of patients during a mean follow-up of 52 months. The 5-year cumulative incidence of metachronous gastric neoplasm was 4.8%. Multivariate analysis showed that moderate/severe corpus intestinal metaplasia and family history of gastric cancer were independent risk factors for metachronous gastric neoplasm development; the hazard ratios were 4.12 (95% confidence interval [CI], 1.23 to 13.87; p=0.022) and 3.52 (95% CI, 1.09 to 11.40; p=0.036), respectively. The methylation level of MOS was significantly elevated in patients with metachronous gastric neoplasms compared age- and sex-matched patients without metachronous gastric neoplasms (p=0.020).

Conclusions

In patients who underwent endoscopic resection of gastric neoplasms, moderate/severe corpus intestinal metaplasia and a family history of gastric cancer were independent risk factors for metachronous gastric neoplasm, and MOS was significantly hypermethylated in patients with metachronous gastric neoplasms.     

Squamous-Cell Carcinoma of the Rectum: A Rare but Curable Tumor

Purpose This study was designed to evaluate one institution’s experience with treatment outcomes for rectal squamous-cell carcinoma. Methods Using our prospective Colorectal Database, we identified patients diagnosed with rectal squamous-cell carcinoma at our institution between 1983 and 2005. Pathology was rereviewed, tumor immunophenotype was compared to control cases of anal squamous-cell carcinoma and rectal adenocarcinoma, treatment modalities and outcomes were analyzed. Results Twelve patients were identified (10 females median age, 58 years). Median distal extent of tumors was 7 (range, 5–8) cm from the anal verge. Treatment included chemotherapy only (n = 1), chemoradiation only (n = 2), induction chemotherapy followed by chemoradiation and surgery (n = 2), chemoradiation followed by surgery (n = 5), and surgery followed by chemoradiation (n = 2). The chemotherapy regimen was 5-fluorouracil-based. Radiotherapy total dose was 50.4 Gy (1.8 Gy/day, daily × 5) external iliac and inguinal nodes were not included in the radiation field. Complete clinical responders to chemoradiation (n = 2) received no further treatment. All seven partial responders underwent surgery; six had complete pathologic response; nodal status in two of six was unknown because they had local excision. Immunophenotypical analysis showed similar keratin expression profile between rectal squamous-cell carcinoma (n = 5) and rectal adenocarcinoma (n = 5), which is different from anal squamous-cell carcinoma (n = 10). All patients were alive without evidence of disease at follow-up (median follow-up, 2.6 (range, 0.5–16) years). Conclusions Our data suggest that most patients treated with upfront chemoradiation therapy followed by surgery did well. Sphincter-preserving surgery is usually feasible. Clinical judgment of tumor response after chemoradiation is not completely reliable. Immunohistochemistry suggests a common cellular origin for rectal squamous-cell carcinoma and rectal adenocarcinoma, which is different from anal squamous-cell carcinoma. Poster presentation at the meeting of The American Society of Colon and Rectal Surgeons, Seattle, Washington, June 3 to 7, 2006. Reprints are not available.     

A Primary Left Atrial Leiomyosarcoma Mimicking Myxoma: Echocardiographic Findings

Echocardiography is the initial imaging modality to delineate the cardiac mass. Clinically echocardiographic information about morphology, location, motion and hemodynamic consequences of the tumor is very important for decision making in terms of additional imaging studies and treatment. We present a case of left atrial leiomyosarcoma masquerading as myxoma preoperatively and discuss the echocardiographic features for distinguishing leiomyosarcoma from a benign tumor, such as myxoma.     

Epithelioid granulomas in chronic hepatitis C: A transient pathological feature

Multiple hepatic granulomas in chronic hepatitis C patients treated with alpha interferon were recently observed. To assess the presence of hepatic granulomas in chronic hepatitis C, and to determine whether their presence is related to interferon therapy or primarily related to chronic hepatitis C viral (HCV) infection, 446 liver biopsy specimens from 239 Japanese patients with chronic hepatitis C were reviewed. Well-formed non-caseating epithelioid granulomas were found in five (1.1%) of 446 liver biopsy specimens from five (2.0%) of 239 patients. All five patients had been followed up for 1 to 3 years, having between one and six liver biopsy specimens taken at intervals of 6 months to 1 year. Four of these five patients received alpha interferon therapy during the follow-up period. Hepatic granulomas were found in one of the pretherapy liver biopsy specimens in four patients and in one of the post-therapy specimens in one patient. Extensive investigation of the aetiology of hepatic granulomas yielded no conclusive findings. The presence of hepatic granulomas could not be demonstrated in follow-up liver biopsy specimens taken from the four patients who had undergone alpha interferon therapy. These findings suggest that hepatic granulomas may appear as an expression of non-specific reaction in HCV-related chronic hepatitis, and are not related to alpha interferon therapy.