Simultaneous occurrence of accelerated nodulosis in lungs, liver, and kidneys, and acute exacerbation of interstitial pneumonia in a patient with rheumatoid arthritis: an autopsy case report.

BACKGROUND: Accelerated nodulosis (ARN) is a rare variant of rheumatoid nodules (RNs) that is characterized by a rapid onset or the worsening of RNs. It generally develops at the fingers in patients with rheumatoid arthritis (RA) receiving methotrexate (MTX). Few case reports have described ARN at an extracutaneous location. CASE PRESENTATION: An elderly patient with long-standing RA was admitted to our hospital with acute respiratory failure. Computed tomography upon admission showed diffuse ground-glass opacities superimposed with subpleural reticular shadowing and honeycombing and multiple nodules in the lungs and liver. Despite the discontinuation of MTX and introduction of an immunosuppressive regimen with pulse methylprednisolone followed by a tapering dose of prednisolone and intravenous cyclophosphamide, the patient died due to the acute exacerbation (AE) of RA-related interstitial lung disease (ILD) following the parallel waxing and waning of a diffuse interstitial shadow and pulmonary and liver nodules. At autopsy, RNs were scattered throughout both lung fields in addition to extensive interstitial changes. RNs were also detected in the liver and kidneys. The foci of cryptococcosis were mainly identified in alveolar spaces. Based on the clinical and pathological findings, these nodules were most consistent with ARN because of acute increases in the size and number of previously detected pulmonary nodules. CONCLUSION: The present case is noteworthy because ARN was concurrently detected in multiple internal organs and may be associated with the AE of RA-related ILD.

[A case of fibrous pseudotumor arising from the paratesticular area].

Intrascrotal fibrous pseudotumor is rarely observed, and only 40 cases have been reported in Japan. A 65-year-old male patient visited this hospital due to a painless right scrotal mass. Computed tomography demonstrated a solid mass with a cystic component ; however, the exact location of the mass in relation to other scrotal contents was not determined. Intraoperative findings revealed a solid mass, 2 cm in diameter, in the tunica vaginalis. The mass could not be dissected from the epididymis ; therefore, high orchiectomy was indicated. A histopathological diagnosis of fibrous pseudotumor was made. The patient's postoperative course was uneventful, and no recurrence has been found. It is difficult to make a diagnosis of paratesticuler tumor based on preoperative radiological results and even macroscopic observation during surgery. We strongly recommend that it is necessary to undergo high orchiectomy for a fibrous pseudotumor in which dissection from the peripheral tissue is very difficult since it may involve various types which may lead to a diagnosis of malignancy.

Abnormalities in chromosome 17 and p53 in lung carcinoma cells detected by fluorescence in situ hybridization.

The value of fluorescence in situ hybridization (FISH) as an aid to deciding the prognosis for lung carcinoma patients, comparing quantitatively the signal from the p53 gene (17p13.1) on chromosome 17, was studied. A dual-labeling technique was used, using probes for the centromeric region of chromosome 17 and for the p53 gene locus. FISH was used on frozen sections of 68 surgically resected lung carcinoma (20 adenocarcinoma; 37 squamous cell carcinoma; 11 large, small, and other cell carcinoma). Hybridization signals were counted for 100-200 interphase nuclei per specimen using a Zeiss confocal laser scanning microscope (Carl Zeiss, Oberkochen, Germany) equipped with a bandpass filter for diaminophenolindole and a longpass filter for rhodamine. Clinicopathologic data were evaluated using the Statistical Analysis System. Chromosome 17 polysomy (three or more signals) was greater in poorly differentiated than in well-differentiated lung carcinoma (P < 0.05). p53 deletion correlated with p53 immunostaining (P < 0.05). Thus, analysis by FISH using DNA probes for chromosome 17 and p53 may be of some, albeit limited, value in determination of prognosis.

Absent inner dynein arms in a fetus with familial hydrocephalus-situs abnormality.

We report a family in which a healthy, unrelated couple had a male fetus with bilateral ventriculomegaly, a normal liveborn girl, a hydatidiform molar pregnancy, a female fetus with ventriculomegaly and situs abnormalities, and a male fetus with hydrocephalus, a three-lobed left lung, and defective tracheal cilia with absent inner dynein arms and a single centriole. A mutation analysis of FOXJ1 and POLL in the last fetus with ciliary defect revealed no mutation within their coding regions. The presence of three affected fetuses of both sexes in a family with phenotypically normal parents suggests that the condition was inherited as an autosomal recessive trait. A thorough evaluation of the thoracic and abdominal situs is recommended before counseling a family of a child with hydrocephalus, because the recognition of situs defects may point to the diagnosis of primary ciliary defect and recurrence risk of 25% for siblings. This figure is much higher than the general risk of 1-2% for siblings of a patient with isolated hydrocephalus.