Extensive ossification in a craniopharyngioma.

A rare extensive ossification occurred in a suprasellar craniopharyngioma of a man who died at 21 years of age. The tumor produced headache, retarded physical and mental development, visual disturbances, and increased intracranial pressure. The neoplasm recurred after surgical and roentgenological treatments. Differentiation of multipotential mesenchymal cells or mesenchymal type cells within the tumor has been suggested as the mode of occurrence of bone in this craniopharyngioma.

Primary mucoepidermoid carcinoma of the intrahepatic bile duct: a case report with review of literature.

A 64-year-old Thai man had a primary mucoepidermoid carcinoma, a variant of the cholangiocarcinoma, of the left lobe of the liver. A 5 x 4 x 3 cm tumor metastasized within the liver and to the porta hepatic and parapancreatic lymph nodes with compression of the head of pancreas and distal part of the common bile duct resulting in obstructive janudice. The patient died within 7 months from massive hemorrhage of a chronic peptic ulcer of the duodenum. Review of the medical literature in the English language disclosed 11 primary mucoepidermoid carcinomas of the bile ducts, including the present instance. Nine tumors were intrahepatic; three were in the right lobe and six in the left lobe. Two neoplasms were extrahepatic; they arose in the common hepatic ducts. There were 6 men and 5 women of 44 to 78 years old; the mean age was 60 years. The size of the tumor ranged from 1.5 to 18 cm in greatest dimension; the average size was 8.4 cm. The tumor metastasized frequently to the regional lymph nodes. Invasion of the portal vein and hepatic artery has also occurred. Ten patients having primary mucoepidermoid carcinoma of the bile duct died within 11 months regardless of treatments. Only one patient was well, 10 months after extensive resection of a 1.5 cm tumor of the common hepatic duct. It is concluded that the prognosis is generally poor for the patient having primary mucoepidermoid carcinoma of the bile duct.

Traumatic neuroma as a cause of obstructive jaundice.

A 70-year-old man with previous cholecystectomy developed progressive obstructive jaundice, 3 months before hospitalization. Intraoperatively, a 2 x 2 x 1.5-cm mass in the distal part of the right hepatic duct was excised to release complete obstruction. It was verified as traumatic (amputation) neuroma. Despite rarity, traumatic neuroma of the bile duct should be considered in patients who have antecedent surgical procedure of the biliary tract with subsequent occurrence of extrahepatic biliary obstruction.

Epithelial-myoepithelial carcinoma of parotid gland: a case report with immunohistochemical and ultrastructural studies.

A 66-year-old man presented with a painless mass of the parotid gland. Light and electron microscopic studies verified the basic nature of the tumor as epithelial-myoepithelial carcinoma, a low-grade malignant neoplasm of the salivary gland. Pathologically, there were two types of cells; the inner eosinophilic epithelial cells lining the ducts and the outer clear cells. The former cells displayed immunoreactivity for cytokeratin and ultrastructural features of apical microvilli and desmosome. The latter cells were positive for actin, S-100 protein, vimentin and the cytoplasm contained actin microfilaments. Such pathological findings were characteristic features of this rare tumor. To our knowledge, this is the first reported case of EMC in Thailand.

Atypical granular cell tumor of the neurohypophysis: a case report with review of the literature.

A 76-year-old man had an atypical granular cell tumor of the neurohypophysis which showed pleomorphic nuclei, mitotic figures, and spindle-shaped cells, extremely rare findings to be encountered. Review of 45 patients with neurohypophyseal granular cell tumor revealed a ratio of 1:2 between male and female with the peak occurrence (31%) in the fifth decade, and with the mean age of 50 years. There were no patients below 20 years of age. The common clinical presentations included visual disturbances and endocrinopathies relating to sex hormones. Surgical removal was the treatment of choice. If it is possible, total extirpation should be attempted. Because of uncertain cellular origin, the lesion should be descriptively diagnosed as granular cell tumor although multiple terms have been proposed.

Massive pulmonary cryptococcosis in an immunocompetent patient.

A 64-year-old man presented with progressive dyspnea. The symptom of severe hypoxia requiring mechanical ventilator, and bilateral pulmonary infiltrates on the chest film led to the clinical diagnosis of adult respiratory distress syndrome. Autopsy demonstrated widespread cryptococci and mucinous material in alveoli with mild inflammatory response.

Macerated fetus in utero relating to intracranial angioma: report of 2 cases.

Two macerated fetuses in utero are presented. The mothers experienced no abnormalities during pregnancy. Both fetuses were found postmortem to have intracranial angiomas. In case 1, the angioma in the choroid plexus of the right lateral ventricle was associated with intraventricular and subarchnoid hemorrhages. There was additional angiomatosis in the leptomeninges and substance of the brain. In case 2, there was leptomeningeal angiomatosis with diffuse subarchnoid hemorrhage. It is suggested that spontaneous rupture of the angioma of the choroid plexus in case 1 and of the leptomeninges in case 2 resulted in immediate death of the fetuses with subsequent maceration in utero. Asymptomatic angioma of the fourth ventricular choroid plexus is also described in case 2 because of its extreme rarity.

Malignant transformation in a benign encapsulated schwannoma of retropharyngeal space: a case report.

Malignant schwannomas are rare in the head and neck, however benign schwannoma of this area are common. Cases of malignant schwannoma have been reported in the nasal cavity, paranasal sinuses, eye, parapharyngeal space and neck with or without an association of von Recklinghausen disease. We described a case of an asymptomatic solitary malignant schwannoma arising in the retropharyngeal space which was treated by local excision and postoperative radiotherapy. To our knowledge, this case may be the first reported case in the world literature.

Hepatocellular carcinoma having progressive dyspnea as clinical presentation.

Hepatocellular carcinoma (HCC) can give multiple different initial clinical presentations. To the author's knowledge, however, dyspnea has not yet been recorded as an initial clinical presentation of HCC. A 39-year-old woman who had dyspnea for 2 months because of HCC is, therefore, reported herein. A chest X-ray revealed reticulonodular infiltration of lungs which led to antituberculous treatment. She died 3 months after initial dyspnea. An autopsy demonstrated a HCC, 1.5 x 2 x 2 cm, at the edge of the left lobe of the liver with widespread intravascular metastases to the lungs. The patient, moreover, had hypertrophy of the right cardiac ventricle being compatible with cor pulmonale. These pulmonary and cardiac findings are regarded to have produced dyspnea in this case.

Primary diffuse leptomeningeal glioblastoma multiforme of brainstem and spinal cord clinically mimicking meningitis: case report and review of literature.

A primary diffuse glioblastoma multiforme affecting the leptomeninges of the brainstem and cervicodorsal region of the spinal cord occurred in a 23-year-old man. Its clinical manifestations mimicked chronic meningitis. Review of 29 primary meningeal gliomas disclosed that 27 gliomas were primary in the leptomeninges, and two were in the dura mater. Twenty gliomas were solitary and 9 tumors were diffuse. There were 17 intracranial, 7 intraspinal, and 5 combined intracranial and intraspinal. About 40 per cent of primary solitary intracranial leptomeningeal gliomas were situated around the lateral fissure of the brain. The most common astrocytoma comprised 48 per cent of primary meningeal gliomas. Other gliomas were less common. The patients' average age was 44 years for both sexes. There was no distinct sexual preponderance in distribution of primary meningeal gliomas.

Angiolipoma of suprasellar region.

An angiolipoma of the suprasellar region occurred in an 8-year-old boy. Review of 5 intracranial angiolipomas, including the current case, revealed that four were in the sellar region of one man and three women. One was in the thalamus of a man. The average age of the patients was 49 years, and average size of the tumors was 2.5 cm across. The angiolipomas associated with the sellar region often created abnormalities of the eye viz exophthalmos, decreased vision, and palsy of the oculomotor nerve. Sudden onset has ensued in a massively hemorrhagic thalamic angiolipoma. The characteristic low density with negative Hounsfield unit values in CT scan and hyperdensity in MRI of the brain suggested adipose tissue component of the angiolipoma. The angiography has depicted the angiomatous component of the latter. The combined characteristic CT scan, MRI, and angiographic features should demonstrate both vascular and adipose tissue elements of the angiolipomas before pathologic examination.

Fatal hemorrhage from additional primary esophageal squamous cell carcinoma in a patient previously having primary bronchogenic adenocarcinoma.

A unique occurrence is presented of additional primary epidermoid carcinoma of the esophagus with fatal bleeding into the upper digestive tract after 3 years of diagnosis of primary bronchogenic adenocarcinoma of an 81-year-old Thai man. The primary bronchogenic adenocarcinoma was surgically removed and followed by radiotherapy and chemotherapy without evidence of tumor recurrence at autopsy. The epidermoid carcinoma of the lower one-third of the esophagus metastasized to the pleura of the remaining right lung. There was no complaint of dysphagia. Outward extension through the esophageal wall rather than intraluminal protrusion of the squamous cell carcinoma was thought to result in the absence of dysphagia. Although it is uncommon physicians should be aware of the occurrence of multiple neoplasms.

Pathologic changes of gut in non-01 Vibrio cholerae infection.

A 14-year-old girl who had beta-thalassemia hemoglobin E disease was infected by bacteriologically proven non-01 Vibrio cholerae at 2 months postsplenectomy and died 37 hours after onset of the malady. Postmortem examination disclosed congestion, edema, and hemorrhagic foci of the mucosa of the small and large intestines. The gut mucosa was focally eroded. The gut wall was infiltrated by leucocytes, especially neutrophils, in all coats representing acute purulent and hemorrhagic enterocolitis. There was hyperplasia of lymphoid follicles in the gut mucosa and lymph nodes. It is suggested that morphologic change of the gut in non-01 Vibrio cholerae infection is more severe than in infection caused by Vibrio cholerae.

Metaplasia of bone in lungs and bronchi: report of 2 cases.

Two cases are reported of pulmonary osseous metaplasia in elderly men aged 91 and 64 years. The first case represented disseminated metaplastic bones occurring throughout the lungs. In the second instance, small foci of metaplastic bones were localized in some bronchial walls of the upper lobe of the right lung which had old treated tuberculosis, severe fibrosis, and bronchiectasis. Both cases also had advanced pulmonary emphysema and interstitial fibrosis. It is suggested that fibrosed interstitium of the lungs and bronchial walls has turned into bony metaplasia in both patients. However, osseous metaplasia from cartilage of the bronchial walls is additionally suggested in case 2. Multiple factors probably play roles in development of bony metaplasia.

Subacute necrotizing encephalopathy (Leigh's disease) in a child with particular reference to CT finding.

An example of subacute necrotizing encephalopathy, the fifth case in Thailand, was recorded. A 7-month-old boy presented clinically with vomiting, lethargy, respiratory difficulty, deteriorated consciousness, and hypotonia. The CT brain scan disclosed bilateral symmetrical radiolucencies in the basal ganglia, especially the lentiform nuclei, and thalami. Postmortem examination of these areas as well as the periaqueductal region revealed subacute necrotizing encephalopathy. It was characterized by necrosis, gliosis, and status spongiosus of the neuropil with relatively preserved neurons, and hyperplasia of small blood vessels as well as endothelium. As far as we are aware, the vast majority of abnormalities in the basal ganglia visualized by CT brain imaging often show calcific foci or high attenuation with asymmetrical distribution. Bilateral symmetrical lesions of low density are rare. We reported such an abnormality in a postmortem proven case of Leigh's disease. To recognize this finding should lead to correct antemortem diagnosis of the latter.

Neurological manifestations in patients with malignant histiocytosis.

Of the 67 patients who were diagnosed with malignant histiocytosis at the Department of Medicine, Chulalongkorn Hospital, from January 1981 to May 1992, seven (10%) were found to have neurological manifestations. In five patients, neurologic diseases were the presenting clinical features. In the remaining two patients, the disease manifested at the time of relapse. Leptomeningeal infiltration, the most common pattern of involvement, was found in five patients. Involvement of the spinal cord was noted in three patients and cerebral parenchyma in two individuals. Two patients demonstrated peripheral neuropathy. Rapid clinical deterioration was observed in all patients after documentation of neurological diseases with a mean survival of 1.8 months. It is concluded that the frequency and pattern of neurological manifestations in patients with malignant histiocytosis are comparable to patients with non-Hodgkin's lymphoma. However, most patients with malignant histiocytosis manifested the neurological symptoms as an initial presentation while in non-Hodgkin's lymphoma, neurologic diseases usually manifest at the time of relapse or disease progression.

Hepatocellular adenoma in a beta-thalassemic woman having secondary iron overload.

An 18-year-old woman had a hepatocellular adenoma of 4 cm across in the right lobe of the liver which was severely hemosiderotic because of beta-thalassemia hemoglobin E disease with numerous blood transfusions. The lesion was an accidental postmortem finding. To our knowledge, this is the second example of liver-cell adenoma occurring in a patient with beta-thalassemia hemoglobin E disease with secondary iron overload. It is suggested that this is an association between hepatocellular adenoma and secondary iron overload of liver cells, a similar event to the relation observed in hepatocellular carcinoma and secondary iron overload of hepatocytes.

Massive cerebral hemorrhage from metastatic hepatocellular carcinoma.

Metastasis of hepatoma to the brain is a rare event. Even rarer is massive hemorrhage of the brain associated with metastatic hepatoma. A 57-year-old man had cirrhosis of the liver with hepatocellular carcinoma. The tumor spread to the lungs and left occipital lobe of the brain. The primary and secondary neoplasms were negative in detection of mucin, but were immunohistochemically positive to cytokeratin CAM 5.2 and KC; the finding supported the hepatocellular origin of the tumor. The metastatic tumor formed papillae in the lung and produced massive hemorrhage in the left occipital lobe. This case raised the total number of intracranial metastatic hepatic carcinomas to 34 cases. Five of 34 hepatic carcinomas metastatic to brain, including the current one, were hepatocellular carcinoma that produced massive hemorrhage.