Retropharyngeal rhabdomyosarcoma mimicking pseudotumor cerebri.

A 6-year-old male presented with headache, vomiting, visual obscuration, and papilledema. Clinical presentation, initial laboratory data, and radiologic evaluation suggested a diagnosis of pseudotumor cerebri. The development of lower cranial nerve palsies after transient resolution of symptoms prompted reassessment of the diagnosis, which revealed retropharyngeal rhabdomyosarcoma involving the right jugular vein. Cerebral angiography demonstrated the complete venous occlusion at the jugular foramen. Careful neurologic follow-up is essential in a patient with pseudotumor cerebri or idiopathic intracranial hypertension.

[An autopsy case of intravascular malignant lymphomatosis with hemophagocytic syndrome, mental confusion and liver dysfunction].

A 66-year-old Japanese man was admitted to Hitachi General Hospital because of fatigue, fever, edema, and icterus. A blood examination showed anemia, leukocytosis, and hyperbilirubinemia. Antibiotics did not alleviate the inflammatory symptoms. On the 13th hospital day, the patient demonstrated mental confusion, with progressive anemia and thrombocytopenia. Bone marrow aspiration revealed an increase of macrophages showing erythrophagocytosis. A diagnosis of hemophagocytic syndrome was made, but no viral infection or hematological malignancy could be detected. The patient was treated with gamma-globulin and methylprednisolone for hemophagocytic syndrome, but died of respiratory insufficiency with progressive hyperbilirubinemia and trombocytopenia on the 29th hospital day, A postmortem examination showed proliferation of lymphoma cells within the small blood vessels of the brain, lungs, liver, and many other organs, but the bone marrow was not involved. An immunohistochemical examination resulted in a diagnosis of intravascular malignant lymphomatosis (IML) of B cell origin. No viral infection was detected with in situ hybridization methods. Although IML occasionally accompanies thrombocytopenia, infiltration of lymphoma cells in the bone marrow is rare. Lymphoma associated hemophagocytic syndrome may cause cytopenia in some patients with IML, as indicated in this case.

Malignant change in a duodenal adenoma in familial adenomatous polyposis: report of a case.

Patients with FAP (familial adenomatous polyposis) are known to be at high risk for duodenal cancer. Although the adenoma-carcinoma sequence is thought to exist in the duodenum, clinical observation of the development of duodenal adenoma to cancer has rarely been reported. We outline a 44-yr-old postcolectomy man with FAP who underwent regular gastroduodenoscopy annually or biannually and was found to be harboring duodenal ampullary cancer 5 yr after colectomy. Endoscopic and pathological examination of the ampullary lesion during these 5 yr revealed progression of pathology from adenoma to carcinoma. Pathology of the surgical specimen confirmed ampullary cancer. This in vivo demonstration of the adenoma-carcinoma sequence highlights the current limitations of duodenal surveillance in FAP.

[Undifferentiated connective tissue syndromes (UCTS) accompanied by laryngeal involvement and autoimmune hepatitis].

Here we report a patient with undifferentiated connective tissue syndromes (UCTS) who developed hoarseness during exacerbation of autoimmune hepatitis. A 51-year-old woman was hospitalized in November 1993 because of hoarseness and liver dysfunction. She had demonstrated Raynaud's phenomenon, polyarthralgia and hoarseness since 1992. In August 1993, liver dysfunction was noted. On admission, laboratory data showed mild leukopenia, thrombocytopenia (WBC 3,900/mm3, platelet 12.4 x 10(4)/mm3), and elevations of transaminase (GOT 96 IU/l, GPT 79 IU/l) and IgG (4,556 mg/dl). Anti-nuclear antibody (ANA) and anti-smooth muscle antibody were positive. Other autoantibodies including anti-DNA antibody, anti-Scl 70 antibody were all negative. LE test and LE cells were also negative. On laryngoscopic examination, lesions that appeared similar to a bamboo-joint were noted at the middle of the bilateral vocal cords. Pathological findings of liver biopsy specimen were compatible with autoimmune hepatitis. She was treated with 30 mg of prednisolone. Polyarthralgia, hoarseness and the abnormalities of the transaminase levels improved rapidly. Laryngoscopic findings were also normalized. We considered this laryngeal involvement to be acute laryngitis accompanied by some UCTS, including a typical systemic lupus erythematosus (SLE) because of arthritis, cytopenia and ANA positivity. Involvement of the larynx in collagen disease is rarely mentioned in published reports.

[Clinicopathological study of diffuse panbronchiolitis--with special reference to relationship between the clinical duration and respiratory bronchiolar lesions].

Fifteen autopsy cases of diffuse panbronchiolitis, with clinical disease duration ranging from 2 months to 56 years since appearance of cough and sputum, were analyzed by reconstruction study using serial sections, with particular regard to the respiratory bronchiolar lesions and clinical duration. In addition, proximal bronchi and bronchioles were examined focusing on dilatation, round cell infiltration and goblet cell metaplasia, and peribronchiolar emphysematous lesions were graded according to the degree of alveolar destruction. Morphological changes of the respiratory bronchioles were classified into five types. Active lesions, with lymphoplasmocytic and xanthoma cell infiltration were classified into three types according to the presence and the size of intraluminal granulation tissue. Scar lesions showing marked fibrohyalinous change were classified into two types, those with and without respiratory bronchiolar stenosis. Active lesions decreased and scar lesions increased with clinical duration. Respiratory bronchiolar lesions were distributed almost equally from the upper lobe to the lower lobe. In these fifteen autopsy cases, the total number of respiratory bronchiolar lesions did not vary with disease duration. Emphysematous lesions increased with clinical duration, and showed a relationship to the presence of scar lesions with stenosis. Bronchiolar dilatation was prominent in cases with a long clinical course. Segmental and subsegmental bronchial dilatation were recognized mainly in the middle, lingula and lower lobes, but there was no relationship between clinical duration and bronchiolar dilatation.

Isolated tricuspid valve stenosis caused by infective endocarditis in an adult: report of a case.

We report herein the case of a 42-year-old man in whom dyspnea on exertion was found to be caused by isolated tricuspid stenosis. Two-dimensional echocardiogram showed thickening of the tricuspid valve with a markedly enlarged right atrium. A color-flow Doppler examination-revealed severe tricuspid stenosis without regurgitation and a Doppler-derived tricuspid diastolic pressure gradient of 23 mmHg. At the time of surgery, the patient was noted to have a stenotic tricuspid valve with thickened leaflets, fused commissures, and almost normal chorda tendineae. The valve leaflets were teased apart to the scattered specimen, and tricuspid valve replacement was successfully performed. Microscopic examination of the specimen demonstrated infective endocarditis. Isolated acquired tricuspid stenosis is extremely rare and, to our knowledge, this is the first case of infective endocarditis being involved as the primary cause.

An autopsy case of intravascular lymphomatosis (neoplastic angioendotheliomatosis) accompanied by high fever, hypertension and without focal sign.

A 75-year-old woman suffered from intermittent high-grade fever and hypertension without any focal sign. Serum lactic dehydrogenase (LDH) was markedly elevated. The fever was resistant to antibiotics and temporarily sensitive to prednisolone. She had heart failure and died. Postmortem examination revealed intravascular proliferation of B lymphocytes, indicative of the diagnosis of intravascular lymphomatosis. The clinical diagnosis is usually very difficult because of the absence of pathognomonic clinical manifestations.