Alveolar Soft Part Sarcoma of the Lung: A Report of Six Cases and Clinicopathological Analysis

BACKGROUND: Alveolar soft part sarcoma (ASPS) accounts for 0.5-1% of soft tissue sarcomas, and often metastasizes to the lung. Cases of pulmonary ASPS of unknown primary site have rarely been reported in literature. METHODS: Here, we report three cases of metastatic pulmonary ASPS and three cases of presumably primary ASPS using immunohistochemistry and clinicoradiologic findings. RESULTS: All of the cases occurred in young females. Two of the cases had metastasized from soft tissue ASPS of the lower extremities, and one case had metastasized from one of the patient? femur bones. Immunohistochemical stains were applied to four cases that had available paraffin blocks. The tumor cells of all cases on which immunohistochemical stains were done were positive for vimentin (4/4, 100%). None of the tumors were positive for myoglobin, desmin, smooth muscle actin, progesterone receptor, estrogen receptor, thyroid transcription factor-1, S-100 protein, pancytokeratin, and HMB-45 antibodies. CONCLUSION: The present study revealed that the rare pulmonary ASPS has nonspecific clinicoradiologic findings. In the immunohistochemical results, no differences existed between the presumably primary ASPS and the metastatic ASPS except for a higher Ki-67 labeling index in the latter (less than 0.1% vs. 30%). The higher index was not dissimilar to those of the extrapulmonary ASPS which showed a tumor with a low proliferation index, signifying a better prognosis and have a low potential to metastasize.

Effect of Intracavitary Injection of Urokinase in the Experimentally Induced Early Pyogenic Liver Abscess of the Rabbit

This study was carried out to assess the effects of intracavitary injection of urokinase in the early liver abscess (ELA) of the rabbits. ELAs were induced on 25 in 47 New Zealand rabbits, which were divided into two groups, with 15 in group A, and 10 in group B. Urokinase was injected into the ELA of group A, and normal physiologic saline into those of group B. One and a half hours after the injections, the rabbits were sacrificed and evaluated by pathologists for the degree of fibrosis of the ELA wall, and fibrinolysis in the ELA itself. Statistical analyses were performed between the two groups. The following ELA sizes for each group were obtained: Group A, 4.3 X 2.9-10.1 X 7.2 mm (mean 7.1 X 4.1 mm); Group B, 4.6 X 2.7-15.0 X 9.7 mm (mean 8.5 X 4.57 mm). Eleven (73%) in group A showed grade II fibrosis of ELA wall, and 8 (80%) in group B showed grade III fibrosis of ELA wall (p=0.002). On pathological analysis, 5 (46%) in group A showed grade II fibrin, and 8 (80%) in group B showed grade III fibrin, of the ELA (p=0.09). In conclusion, injection of urokinase, into the ELAs, can reduce the degree of fibrosis of abscess walls.

Fibromatosis of the Parotid Gland: A Case Report

A 51-year-old woman was presented with a palpable mass in the infraauricular area that had existed for 4-months. The mass from the superficial lobe of the parotid gland was 2.7 cm in the greatest dimension and was ill-defined with rubbery, homogeneous, and fibrotic appearance. Histologically, the lesion was infiltrative into the lobules of the paratid gland, and was composed of a proliferation of spindle or stellate cells with cellular and sclerotic areas. On immunohistochemistry, the cells were only positive for vimentin and focally for smooth muscle actin. Differential diagnosis from nerve sheath tumors and solitary fibrous tumors involving the parotid gland was emphasized.

Disseminated Systemic Candidiasis and Aspergillosis in a Liver Transplant Patient: An Autopsy Report

Postoperative complications of liver transplantation include rejection, infection, hepatic vascular thrombus, and primary graft failure, etc. Among them, fungal infection shows nonspecific clinical symptoms and overlapping laboratory findings with variable etiologies causing post-transplant hepatic dysfunction. Therefore, early diagnosis of fungal infection is not easy. Here, we report an autopsy case of disseminated candidiasis and aspergillosis in a liver transplant patient. The case was at first misinterpreted as acute cellular rejection on biopsy because the histology of predominantly cellular infiltration, ductulitis and endothelialitis were similar to those of acute cellular rejection. On autopsy, the liver, lung and kidney showed multifocal hemorrhagic infarcts due to intra-arterial fungal emboli, which were composed mostly of candida species and a minor fraction of aspergillus. Fungal thrombi invading portal vein, intrahepatic arterioles with subsequent coagulation necrosis, venulitis and ductulitis were ascribed to the misdiagnosis on biopsies. It is unusual that systemic candidiasis, unlike aspergillosis, involves large arteries.

Fatal Strongyloidiasis with Residual Cutaneous Larvae: An Autopsy Case Report

Strongyloides stercoralis hyperinfection in immunocompromised patients is difficult to control due to delayed diagnosis, especially in nonendemic areas. A 70-year-old diabetic woman came in with an intermittent diarrhea. She received massive steroid therapy for a week under the impression of idiopathic gastrointestinal eosinophilic syndrome. Diagnosis of strongyloidiasis was made by demonstration of filariform larvae in two repeated sputum specimens two months later. Despite massive administration of albendazole, she died of diabetic ketoacidosis and septic shock. Autopsy revealed a diffusely thickened bowel wall, superimposed invasive pulmonary aspergillosis and the near total destruction of the lungs. Filariform larvae were detected only in the cutaneous lesions. It was presumed that Strongyloides stercoralis hyperinfection was caused by autoinfection in the patient's immunosuppressed status. The present case has two clinical importances; first, intensive treatment with albendazole could erradicate parasites except for the skin but sepsis presumably occurred due to a hypersensitivity reaction against liberated endotoxins during the death of the parasites by the drug. Second, cutaneous parasitic lesion resists the intensive antihelminthic treatment in an immunocompromised host.

IL-12 and TNF-alpha productions from human peripheral blood mononuclear cells in untreated patients with active pulmonary tuberculosis stimulated with 30-kDa or TSP antigen of Mycobacterium tuberculosis H37Rv

No abstract available.

Cystadenoma of the Rete Ovarii

The rete ovarii is a rare site for tumor development. We present a case of cystadenoma originating from the rete ovarii of a 58-year-old woman. It was a unilocular cyst of 7.0 cm in size, lined by flat, cuboidal, or columnar epithelial cells supported by fibromuscular tissue. Papillary growth and crevice formation of the inner surface were characteristic. The presence of a normal rete ovarii was helpful in correct diagnosis.

A case of retroperitoneal lipoleiomyoma

We report a case of lipoleiomyoma which arose in retroperitoneum and presented with progressively distended abdomen in a 56-yr-old woman. The tumor was well encapsulated and consisted of two components, benign smooth muscle cells and mature adipose tissue without any atypia. It is likely to be mistaken as extrarenal angiomyolipoma, well-differentiated liposarcoma and leiomyoma with fatty change. We review the histologic characteristics of previously reported myolipoma and describe essential points of differential diagnosis.

Vascular Endothelial Growth Factor and basic Fibroblast Growth Factor Expression in Early Gastric Carcinomas: Correlation with Clinicopathologic Factors / 대한암학회지

PURPOSE: Recent studies have demonstrated that angiogenesis and its inducers such as vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) play an important role in growth, progression, and metastasis in gastric carcinomas. In this study, the authors investigated the prognostic significance of angiogenesis, VEGF, bFGF with respect to conventional clinicopathologic factors in early gastric adenocarcinomas. MATERIALS AND METHODS: Sixty-six specimens resected from patients with early gastric carcinomas were investigated by immunohistochemical staining with antibodies against VEGF, bFGF, and CD31. RESULTS: In this study, high expression rates of VEGF and bFGF as well as high level of angiogenesis were observed. In addition, the expression rate of VEGF was correlated well with angiogenesis. However, the clinicopathologic factors, such as age, sex, location, growth pattern, lymph node metastasis, submucosal invasion, and degree of differentiation, were not significantly associated with the expression of VEGF and bFGF, and angiogenesis. CONCLUSION: These results suggest that controlling angiogenesis and its inducers might be a therapeutic target rather than a prognostic factor in early gastric carcinomas.

Sex Cord Tumor with Annular Tubules and Serous Surface Papillary Carcinoma of the Ovary: A case report

Sex cord tumor with annular tubules (SCTAT) is a rare ovarian neoplasm which usually occurs in two forms. In patients associated with Peutz-Jeghers syndrome, the tumors are usually small, bilateral or multifocal, and show benign clinical course. However, tumors from patients without the syndrome are often large, usually unilateral, and rarely show malignant behavior. Serous surface papillary carcinoma (SSPC) is an aggressive neoplasm which involves peritoneal linings, including ovarian surface. Recently, we encountered a case of an unusual combination of SCTAT and SSPC in the ovary of a 55-year-old Korean woman presented with abdominal distention for one year. Systemic review and physical examination were within normal limit, except for abdominal discomfort and distention. There was no stigmata of Peutz-Jeghers syndrome in all diagnostic examinations, including gastroscopy and colonoscopy. Pelvic computed tomography showed adnexal mass with multiple peritoneal nodules. Exploration revealed uterine and ovarian surfaces covered with multiple, yellow-white papillary nodules. However, the sizes of both ovaries were within normal limit. Typical serous papillary carcinomas were identified in nodules from peritoneum and ovarian surfaces. Well-circumscribed columnar epithelial cell nests composed of ring-shaped tubules encircling hyalinized basement membrane-like materials were found in the ovary away from serous surface papillary carcinoma.

A Clinicopathological Study of Posttransplant Liver Biopsy

Liver biopsies are used routinely in the assessment of graft dysfunction following liver transplantation and generally considered to be the most reliable method for the diagnosis of posttransplant complications with overlapping clinical and laboratory findings. To investigate posttransplant complications causing graft dysfunction and usefulness of liver biopsy, we analysed clinicopathologic features of 65 posttransplant liver biopsies, 2 autopsies and an explanted liver, taken from 20 patients. The frequencies of posttransplant complications were acute cellular rejection in 9 patients (45%), postoperative infection in 11 patients (55%), of which cytomegalovirus (CMV) infection and systemic invasive aspergillosis with candidiasis occured in 10 patients (50%) and 1 patient (5%), respectively. Remainders were hepatic arterial thrombosis in two (10%), primary graft dysfunction due to fatty donor liver in one (5%), and posttransplant lymphoproliferative disorder (PTLD) in two (10%). There were no chronic rejection or recurrent disease. Postoperative mortality was 25%. Histologic grade by Banff schema was well correlated with clinical parameters associated with unfavorable short term prognosis. CMV infection was associated with acute cellular rejection in 6 out of 10 patients (60%). Immunohistochemical staining for CMV was more sensitive method than CMV in situ hybridization or histologic detection of viral inclusion on tissue section. It was unique that one case of PTLD developed under the circumstances of the lowest dosage of immunosuppression and took grave outcome. Based on these results, we concluded that clinicopathologic correlation with integration of all the clinical and laboratory findings is necessary in the interpretation of accurate and early diagnosis of posttransplant liver biopsies. The interrelationship between chronic rejection and CMV infection as well as pathogenetic factors of PTLD remains to be clarified through further ongoing observation.

Chondroblastoma-like Extraskeletal Chondroma: A case report

Extraskeletal chondromas are relatively uncommon benign cartilaginous tumors of the soft tissue and well known to pose a considerable diagnostic problem because of histological variations including the immature appearance of their tumor cells. Recently, we have experienced a case of extraskeletal chondroma mimicking benign chondroblastoma. The patient was a 47-year-old woman who complained of a painful subcutaneous swelling on the radial aspect of 4th proximal interphalangeal (PIP) joint in the left hand for 6 months. Radiologic examination of the 4th finger revealed a 1cm-sized soft tissue mass. Histologically, the tumor was characterized by a lobulated mass which was composed of dense proliferation of chondroblast-like cells admixed with a few multinucleated giant cells of osteoclastic type. However, there were focal areas of typical chondroma which showed lace-like intense calcification around the differentiated chondrocytes.

Kaposiform Hemangioendothelioma Complicated by Kasabach - Merritt Phenomenon with Bone Involvement in an Adult / 대한암학회지

Kasabach-Merritt phenomenon does not occur with common hemangioma, rather it is associated with the more aggressive Kaposiform hemangioendothelioma and rarely with other vascular neoplasm. We report the case of an adult who was diagnosed as Kaposiform hemangioendothelioma complicated by Kasabach-Memtt phenomenon. This is the first report in Korea of an adult with Kasabach-Merritt phenomenon who has osteolytic changes of femur, pelvic bone, and lumbar spine.

Alveolar Soft Part Sarcoma of the Lung Diagnosed by Fine Needle Aspiration Cytology: A Case Report / 대한세포병리학회지

Alveolar soft part sarcoma(ASPS) is a rare malignant neoplasm with a distinct clinicopathologic entity of which fine needle aspiration(FNA) cytologic findings have been described in only a few reports. Although patients usually present with an isolated soft-tissue mass in the extremity, metastasis can occur in about 13 % of total cases and the most frequent metastatic site is the lung. We have recently experienced a FNA cytologic case of ASPS in the lung. A 23-year-old female patient was admitted to this hospital due to 2-month-history of cough. She had been good in health before the visit. Chest computed tomography revealed multiple, variable sized, bilateral pulmonary nodules. Physical examination and other staging work up revealed no other lesions except for pulmonary nodules. A percutaneous transthoracic FNA was performed from the pulmonary nodules. The smear was cellular and most cells were arranged singly. In addition, a few clusters lined by thin-walled vasculature with a pseudoalveolar pattern were present. Some of the tumor cells were large and polygonal to oval with abundant granular or vacuolated cytoplasm. Most cells were naked nuclei showing finely granular chromatin pattern with prominent central nucleoli.

Persistent Truncus Arteriosus with Survival to the Age of 29 Years

The Truncus arteriosus is a congenital malformation in which only one great artery arises from the base of the heart and gives origin to the systemic, pulmonary and coronary arteries proximal to the aortic arch. Pulmonary blood flow is governed by the size of the pulmonary arteries and the pulmonary vascular resistance. In infancy, pulmonary blood flow is usually excessive because pulmonary vascular resistance is not greatly increased. Thus, despite an obligatory admixture of systemic and pulmonary venous blood in the common trunk, only minimal cyanosis is present. Rarely pulmonary blood blood flow is restricted by hypoplastic or stenotic pulmonary arteries arising from the truncus. The prognosis in persistent truncus arteriosus is very poor. the median age of survival of the 94 patients with the disease reported up to 1962 was only five weeks. The longest survival reported is the case of the man described by Carr et al who lived to the age of 36 years and 2 months. We report the case of a man with persistent truncus arteriosus who lives to the age of 29 years.

A Case of Isolated ACTH Deficiency Accompanied by hyperprolactinemia / 대한내분비학회지

Isolated ACTH deficiency is a uncommon disorder causing secondary adrenocortical insufficiency. Less than 200 cases have been reported in the world. The major clinical manifestations are hypoglycemia, weight loss, hypotension, anemia, weakness, nausea, inability to excrete water load, and hyponatremia. A 56-year-old male was admitted because of weakness, fatigue, nausea and vomiting. He was pallor and not associated with hyperpigmentation. The basal plasma ACTH and cortisol levels were 8.30 pg/ml and 0.6 ug/dl. The serum cortisol did not response to rapid ACTH stimulation test. On combined pituitary stimulation test, the cortisol did not response to insulin-induced hypoglycemia. Other anterior pituitary hormones showed normal responses except elevated prolactin level. Six months after glucocorticoid replacement therapy, the elevated basal prolactin level returned to normal. Brain MRI did not show any anatomic abnormalities of the sellar and suprasella area. We report a case of isolated ACTH deficiency accompanied by hyperprolactineia, which respond to glucocorticoid replacement therapy.

A Case Report of Hypokalemic Periodic Paralysis with Arrhythmia

The hypokalemic periodic paralysis is characterized by intermittent falccid paralysis of extremities with spontaneous recovery. It is rarely accompanied by cardiac arrhythmia, especially fatal ventricular tachycardia or torsades de pointes. We observed a 29 year old man, who had suffered from intermittent periodic paralysis and fatal ventricular tachyarrhythmia. He had the first episode of muscle weakness in his low grade of elementary school, which lasted for 20 -30 hours. Similar episodes of muscle weakness occurred 1 -7 times per year, especially after carbohydrate rich food. On admission to emergency room, his chief complaints were generalized weakness and chest tightness, serum potassium level was 1.6mEq/l, and four extremities showed Grade 0 motor weakness. His electrocardiography(ECG) showed Atrioventricular dissociation due to sinus tachycardia and accelerated junctional rhythm, intraventricular conduction distrubance. During intravenous potassium administration, ECG showed sustained ventricular tachycardia and cardiovascular collapse occurred. So we carried out resuscitation and cardioversion. After resuscitation, he recovered from cardovascular collapse and ECG showed sinus tachycardia. But during continuous monitoring ECG showed torsades de pointes with cardiovascular collapse. We carried out resuscitation and defibrillation repeatedly. Serum potassium level was 1.7 - 1.8mEq/L at that time. After successful resuscitation, ECG showed sinus rhythm, and his mental status was fully recovered. After he admitted to intensive care unit, paralytic attack and cardiac arrhythmia did not occurred any more. Serum potassium level was maintained between 3.9 -6.1lmEq/L during his hospital days. He was fully recovered but could not take any medications(e.g. acetazolamide, potassium supplying agent and antiarrhythmic drugs) due to severe gastrointestinal disturbances. During the 30 months of postdischarge period, he experienced three mild paralysis attacks, but they were not accompanied by chest tightness, palpitation or syncope.