KIT and platelet-derived growth factor receptor α gene expression in laryngeal small cell carcinoma.

OBJECTIVE: Small cell carcinoma has the worst prognosis of all laryngeal neoplasms. In order to further characterise this tumour, with a view to development of new therapeutic approaches, we report the results of KIT gene and platelet-derived growth factor receptor α gene expression analysis, for two extremely rare cases of primary small cell carcinoma of the larynx. METHOD: Case reports, including immunohistochemical study, and review of the literature. RESULTS: We present two patients with laryngeal small cell carcinoma, who died from tumour metastasis to the lungs and brain despite aggressive treatment. Immunohistochemical studies revealed positive reactions for KIT gene expression and platelet-derived growth factor α gene expression in patient one, and for KIT gene expression in patient two. Molecular genetic analysis, using polymerase chain reaction direct sequencing, identified no mutations of the KIT or platelet-derived growth factor receptor α genes. CONCLUSION: Although further investigation is necessary regarding KIT gene expression and platelet-derived growth factor receptor α gene expression in laryngeal small cell carcinoma, the reported results suggest that these genes may be significant in the development of molecular targeted therapy.

[Mucinous bronchiolo-alveolar cell carcinoma with marked serum elevation of CA19-9: report of a case].

An 83-year-old woman was referred to our hospital to examine for an infiltration shadow in the right lower lobe with progressive bronchorea Computed tomography showed an infiltration lesion with the longest diameter of 10 cm in the right lower lobe and a tumor with the longest diameter of 3 cm in the middle lobe. Serum level of carbohydrate antigen (CA) 19-9 markedly increased to 37,670 U/ml over a period of 3 months. The pathologic study obtained by a transbronchial tumor biopsy revealed a mucinous adenocarcinoma The patient underwent video-assisted thoracoscopic right middle and lower bi-lobectomies with nodal sampling. Postoperative course was uneventful Pathologic study revealed an adenocarcinoma with mixed subtypes, predominantly composed of mucinous bronchiolo-alveolar cell carcinoma (BAC). Immunohistochemical study showed CA19-9 positivity in the apical surface of some tumor cells and diffuse patterns of other tumor cells. Postoperative course was uneventful and serum CA19-9 levels decreased within the normal range. Clinico-pathologic features of the lung cancer patients with serum elevation of CA19-9 and CA19-9 positivity in the cancer cells was discussed. CA19-9 can be an useful tumor marker in the selected patients with mucinous BAC.

[Hemoptysis one year after video-assisted thoracoscopic bullectomy for spontaneous pneumothorax: report of a case].

A 63-year-old man who had underwent video-assisted thoracoscopic bullectomy for left spontaneous pneumothorax 1 year before developed recurrent hemoptysis. Chest computed tomography showed previous stapling of the subsegmental bronchus in the left apico-posterior segment Bronchial arteriography showed hypervascularization of bronchial artery in the left upper segment and pooling of contrast medium along the staple-suture line. Video-assisted thoracoscopic apico-posterior segmentectomy was performed successfully. Pathological examination revieled hemosiderin lining along the surgical stump of B(1+2)cii, neither with infection nor infarction. These findings suggest that mechanical stapling of B(1+2)cii induced ischemia in the peripheral lung parenchyma causing bronchial hypervascularization. Late onset hemoptysis should be kept in mind as a complication after bullectomy with a mechanical stapler.

[Massive hemoptysis due to traction bronchiectasis caused by thoracic radiotherapy 23 years earlier].

We reported a 72-year-old woman who had a massive hemoptysis due to traction bronchiectasis in the left upper lobe. The patient underwent left radical mastectomy followed by thoracic radiotherapy for left breast cancer. The chest computed tomography showed traction bronchiectasis in the atrophic left upper lobe and the bronchial angiography showed hypervascularization of bronchial and internal thoracic arteries to the left upper lobe. Left upper lobectomy was performed after bronchial embolization for recurrent massive hemoptysis. Postoperative course was uneventful. Pathologic findings showed non-anatomical but clearly-bordered traction bronchiectasis with hypervascularized bronchial artery in the left upper lobe. These lesions were consistent with the field of the previous radiotherapy. Traction bronchiectasis causing massive hemoptysis should be considered as one of pulmonary sequelae after thoracic radiotherapy.

A case of inflammatory fibroid polyp causing small-bowel intussusception in which retrograde double-balloon enteroscopy was useful for the preoperative diagnosis.

We showed a newly developed method, retrograde double-balloon enteroscopy, to be useful for preoperative diagnosis in a case of inflammatory fibroid polyp accompanied by small-bowel intussusception. A 64-year-old woman was admitted to our hospital with small-bowel intussusception. Results of radiographic and ultrasonographic examination were suggestive of a small-bowel mass. Retrograde double-balloon enteroscopy was performed in an attempt to make a preoperative diagnosis. Endoscopic observation, in combination with histological findings derived from endoscopic biopsy, was suggestive of an inflammatory fibroid polyp. The patient then underwent laparotomy with minimal incision, which revealed a polypoid mass leading to a jejunojejunal intussusception, without bowel necrosis, and a partial small-bowel resection was performed. The pathological diagnosis was an inflammatory fibroid polyp.

[A clinicopathological evaluation of prognostic factors of urothelial tumors of the renal pelvis and ureter].

We investigated the clinicopathological features of 109 patients with urothelial tumors of the renal pelvis and ureter who underwent surgery at four institute from April, 1975 to September, 1997. The patients consisted of 71 males and 38 females, and the mean age was 66.8 years, ranging from 41 to 92 years. Mean followup period was 43 months. The prognostic significance of the pathological factors; pT, Grade, INF, tumor size, pL, pV and pN were evaluated. All these factors affected the survival rates significantly in univariate analysis. Multivariate analysis showed the most influential prognostic factors to be pT and pN.

Anatomical segmentectomy of the head of the pancreas along the embryological fusion plane: a feasible procedure?

BACKGROUND: Less extensive resection of the head of the pancreas has been the procedure of choice recently for low-grade malignant neoplasms. The anatomical detail of the head of the pancreas is currently insufficient for segmental resection along the embryological fusion plane. METHODS: The anatomy of the head of the pancreas was analyzed in 31 consecutive autopsy specimens. An anterior (n = 10) or posterior (n = 10) segmentectomy of the head of each pancreas was performed along the macroscopically found fusion plane. The pancreatic arteries, the portal vein, the bile duct, and the pancreatic duct were visualized by injecting 3 silicon dyes of different colors. Another 11 specimens were examined by pancreatography before and after anterior (n = 5) or posterior (n = 6) segmentectomy. Eight of these 11 specimens were stained immunohistochemically to reveal the distribution of pancreatic polypeptide cells after segmentectomy. RESULTS: The cleavage between the anterior and posterior segments was discovered at the anterior inferior edge or at the posterior superior edge of the head of the pancreas. Anterior segmentectomy was accomplished while preserving the anterior and posterior pancreaticoduodenal arcades and the lower bile duct in the posterior segment. Posterior segmentectomy involved the removal of the lower bile duct and the posterior pancreaticoduodenal arcades. Pancreatography after segmentectomy showed the division of the ducts of Wirsung and Santorini with the peripheral branches. The immunohistochemical boundary of pancreatic polypeptide cells coincided with the surgical plane. These results showed the anterior and posterior segments were originated from the embryologically dorsal and ventral primordia, respectively. CONCLUSIONS: The current anterior or posterior segmentectomy of the head of the pancreas corresponded to the resection of the embryologically dorsal or ventral primordium, respectively. Anterior segmentectomy of the head of the pancreas might be a clinically applicable procedure; however, posterior segmentectomy involving the resection of the lower bile duct may be impractical.

Anterior tributaries of the portal vein at the superior margin of the pancreas: is "tunneling" procedure safe during pancreatic surgery?

BACKGROUND: It is widely believed that using the "tunneling" procedure during pancreatoduodenectomy is a safe maneuver because the anterior tributaries of the portal vein (PV) are absent or very rare. METHODS: The head of the pancreas with the duodenum and the common bile duct (CBD) was obtained from 22 autopsy cases. Three colors of silicon-polymerase dyes were injected into the pancreatic artery, PV, and bile duct. The tributaries of the PV at the superior margin of the pancreas were studied. RESULTS: There was no anterior branch of the intrapancreatic PV. The anterior superior pancreaticoduodenal vein (ASPDV) gave a definite branch to the anterior aspect of the PV at the superior margin of the pancreas in 4 of 22 cases. When the posterior superior pancreaticoduodenal vein (PSPDV) was located in front of the CBD (5 of 22 cases), the ASPDV and PSPDV had a thick common trunk at the right side of the PV. In these 5 cases, no individual branch from the ASPDV was found at the anterior aspect of the PV. CONCLUSION: PV sometimes has a definite anterior tributary at the superior margin of the pancreas; this must be kept in mind to perform the tunneling procedure during pancreatic surgery.

[Pancreas like acinar glands in the esophagogastric junctional mucosa].

Esophagogastric material resected for malignancy, obtained from 52 adult patients, was examined histologically. In a longitudinal examination of 32 total gastrectomies, intramucosally located pancreatic acinar cell-like cells (PALC) were observed frequently (10 of 12 patients, p < 0.05) only in the uppermost one-sixth part, especially in a 10 mm-long zone around the squamocolumnar junction (SCJ). Entire sampling of the junctional mucosa revealed no significant difference in frequency of occurrence of PALC between esophageal and gastric cancer groups (13 of 20 patients, 20 of 31 patients, respectively). In 59 sections examined precisely, 43 sections showed zonal atrophy of the gastric glands. More than half of these zones (25 of 44 zones) did not overlap with 4 mm-long SCJ zones. In the latter zones, stratified columnar epithelia, epithelial islands, and PALC were observed more frequently, although intestinal metaplasia was not. These data suggested that the occurrence of PALC is correlated with the anatomical site, i.e. SCJ, but not with such ill conditions, i.e. atrophic gastritis or gastric cancer. PALC in the SCJ may be orthotopic.

Elastic fiber degeneration in Costello syndrome.

Clinical and pathological observations of a 6-month-old-boy with Costello syndrome are reported. The main clinical findings were loose skin of the neck, hands, and feet, deep palmar and plantar creases, typical "coarse" face with thick lips and macroglossia, relative macrocephaly, mental retardation, short stature, arrhythmia, large size for gestational age, and poor feeding. At age 6 months he died of rhabdomyolysis. The major pathological findings were fine, disrupted, and loosely-constructed elastic fibers in the skin, tongue, pharynx, larynx, and upper esophagus, but not in the bronchi, alveoli, aorta, or coronary arteries. Hyperplasia of collagen fibers in the skin, hyperplasia of the mucous glands in the bronchus, narrowing of the pulmonary artery, degeneration of the atrial conduction system, calcification and ballooning of skeletal muscle fibers with infiltration of macrophages, and myoglobin depositions in the collecting ducts in the kidney were also observed. The degeneration of elastic fibers was confirmed in the skin of a second Costello syndrome patient. Expression of elastin mRNA in the patient's fibroblasts was normal in size and amount. Given that elastic fiber degeneration was observed in the tissues with clinical symptoms, we speculate that a defect of elastic fibers, possibly relating to alternative splicing in the elastin gene or to defects in elastin microfibrils, might be involved in the pathogenesis of Costello syndrome.

A case of cerebral endometriosis causing catamenial epilepsy.

We present the second reported patient with cerebral endometriosis. She had repetitive partial seizures on the first day of her menstrual cycle. The seizures were controlled by danazol after the causative lesions were surgically removed.