Juvenile and adult xanthogranuloma. A histological and immunohistochemical comparison

Thirteen cases of juvenile xanthogranuloma (JXG) and 13 cases of adult-type xanthogranuloma (AXG) were compared at the light and immunohistochemical levels. Histologically, four main cell types (vacuolated, xanthomatized, spindle-shaped, and "oncocytic") were seen in variable proportions (from monomorphous to mixed variants) with different types of giant cells (nonspecific, foreign body, Touton, and "ground-glass"). Giant cells were more prominent in AXG than in JXG; oncocytic cells (characterized by an eosinophilic, slightly granular cytoplasm similar to thyroid oncocytic cells) and mostly periodic acid-Schiff (PAS) negative giant cells with a ground-glass appearance (6 of 26) were not observed in classic JXG (i.e., occurring in children < 2 years old). Immunohistochemically, JXG and AXG gave similar results: most xanthogranuloma cells labeled strongly with KiM1P and vimentin, while HHF35 and HAM56 stained less intensively. Factor-XIIIa (FXIIIa), KP1 (CD68), and HAM56 stained mostly in the periphery of the lesions. Some markers gave variable results: peanut agglutinin (PA), 60%; alpha-1-antitrypsin, 50%; lysozyme, 25%; LN3 (HLA-DR), < 10% of cells positive. Others were negative: S-100, MAC387 (L1 antigen), LeuM1 (CD15), desmin, smooth muscle-specific actin, and QBEND10 (CD34). This profile helps to delineate xanthogranuloma from histological stimulants such as dermatofibroma (which is FXIIIa+, LN3+, KP1-, and PA-) and multicentric reticulohistiocytosis (which is FXIIIa-, KP1+, PA-, and HHF35-).     

Juvenile thyroid carcinoma with metastasis to the bilateral cervical lymph nodes--a case report

A case of juvenile thyroid carcinoma with metastasis to the bilateral cervical lymph nodes in a 9-year-old male is reported. The clinical picture of juvenile thyroid carcinoma is characterized by early metastasis to the lungs and cervical lymph nodes. In Europe, there have been many reports of thyroid carcinoma after radiation. However, our patient had received no radiation. Surgery consisted of subtotal thyroidectomy and right modified neck dissection. The tumor was a papillary adenocarcinoma and metastasis was seen in 24 out of 38 lymph nodes removed. The serum thyroglobulin level, determined by radioimmunoassay, was 184 ng/ml preoperatively, but by 8 months postoperatively the level fell to 48 ng/ml. No signs of recurrence have been found to date (30 months after the operation).     

Necrobiotic xanthogranuloma with cardiac involvement

A patient with necrobiotic xanthogranuloma (NXG) and paraproteinaemia, who was followed-up for several years, and treated with low-dose chlorambucil, died as a result of a respiratory illness. The significant findings at autopsy were a xanthogranuloma of the spleen and giant cell myocarditis. The myocardial lesions were composed of macrophages, giant cells and lymphocytes. This finding is important because four of five known autopsied patients with NXG have had giant cell myocardial disease, and an effort at antemortem diagnosis should be made.     

Spontaneous hyphema associated with ingestion of aspirin and ethanol

Unilateral hyphema, hematuria, and ecchymoses developed in a previously healthy 42-year-old women after the ingestion of aspirin and ethanol. There was no evidence for ocular trauma, disease, or vascular malformation by slit-lamp examination and gonioscopy. Platelet count and coagulation tests were normal. The patient's bleeding time was prolonged and there was impaired platelet aggregation. Delayed (secondary) aggregation in response to collagen, adenosine diphosphate, and epinephrine was decreased, as was aggregation induced by thrombin and serotonin. These data indicate that the qualitative platelet defect was induced by both aspirin and ethanol. Anterior chamber hemorrhage subsided after discontinuation of aspirin and ethanol, and the hyphema subsequently resolved. Bleeding time and platelet aggregation were normal two weeks after the patient's initial presentation. A prolonged bleeding time in association with normal platelet count, prothrombin time, and partial thromboplastin time indicated a qualitative platelet defect, which is most commonly drug-induced. Defective platelet function resulted in spontaneous hyphema.     

A case of xanthogranuloma of the urinary bladder

A case of xanthogranuloma of the urinary bladder is reported. A 68-year-old man was referred to our hospital for the evaluation of microscopic hematuria. At that time, he did not have an abdominal tumor and ultrasonography showed no abnormality of the kidneys and the bladder wall. Two months later, he was admitted with the chief complaints of perineal discomfort and non tender fist size mass was palpable in the lower abdomen. Ultrasonography, computed tomographic scan and magnetic resonance imaging MRI demonstrated a supravesical mass which was strongly suspected as urachal tumor. Total cystectomy with urachal resection was performed. The histological diagnosis was xanthogranuloma. The patient has been in good health without recurrence, 4 years after surgery. We discuss xanthogranuloma of the urinary bladder in the literature.     

Intra-operative hyphema: a complication of retinal detachment surgery with the Choyce pseudo-phakos

During retinal reattachment surgery in an eye with a Choyce pseudo-phakos a large hyphema developed. The probable mechanism was rupture of blood vessels in the angle of the anterior chamber during drainage of sub-retinal fluid as all the eye structures collapsed around the rigid pseudo-phakos.     

Colloid cyst xanthogranuloma of the third ventricle--a case report

Diagnosis and treatment of malignant pericardial effusion are difficult in clinical practice. This paper reports the feasibility and reliability of percutanous trans-catheter intervention for the diagnosis and treatment of malignant pericardial effusion. Under echocardiagraphic guidence, pericardial puncture was performed and a heamostasis sheath was placed in situ by Seldinger technique. Through the sheath multiple biopsies of the pericardium were taken for pathological examination. A pigtail catheter was inserted to drain off the effusion. According to pathological report suitable anti-cancer drugs were instilled into the pericardial cavity through indwelling catheter for 3-5 days. The catheter was withdrawn when fluid did not re-accumulate. Diagnosis was made in over 96.0% of the cases. Upon follow-up for 3-6 months, complete remission was seen in 96.0% of the treated patients. No significant complication was found in all patients. Percutaneous trans-catheter intervention is a useful approach for the diagnosis and treatment of malignant pericardial effusion.     

Adenocarcinoma of the iris pigment epithelium

We treated an 81-year-old man with a pigmented lesion of his right iris. The lesion was locally excised. Light microscopic examination, including immunohistochemistry, and electron microscopic examination were performed. The diagnosis was low-grade adenocarcinoma arising from the iris pigment epithelium. Adenomatous proliferations of the iris pigment epithelium are exceedingly rare and have not been shown to metastasize. Local resection is recommended if the lesion exhibits growth.     

Corneal sensitivity in people with the same and with different iris color

Corneal touch thresholds were determined for both eyes of 15 people with different colored irides (a condition known as heterochromia) and of 25 people with similar colored irides. It was found that corneal sensitivity is not significantly different in both eyes of either group. Therefore it is suggested that the fact previously demonstrated that corneal sensitivity is related to iris color is not of corneal origin.     

A case of xanthogranuloma of the urinary bladder following herniorrhaphy]

We report a case of xanthogranuloma of the bladder following herniorrhaphy. The patient complained of urinary frequency, residual feeling, and presented with a solid mass located on the dome of the bladder. He had undergone previous inguinal herniorrhaphy. Transurethral biopsy revealed an inflammatory change. Partial cystectomy was carried out for en bloc removal of the tumor. The histological diagnosis was xanthogranuloma of the bladder. Postoperatively, the symptoms disappeared. This is the sixth case report of xanthogranuloma of the urinary bladder and the first case following herniorrhaphy in the Japanese literature.     

Juvenile arthritis with oligoarticular onset

Patients with oligoarticular onset of juvenile arthritis form a large group that is heterogeneous with regard to clinical presentation, further evolution and outcome. The three established subgroups do not cover the whole patient population and are not always easily distinguishable at onset. Therefore, the outcome of children with oligoarticular onset is still, on the whole, unpredictable. Treatment has been very conservative, but, as part of it, the use of intra-articular corticosteroids is increasing and should be encouraged. The question of whether to give slow-acting anti-rheumatic drugs is a difficult one, as these have neither been studied nor recommended for use in persistently oligoarticular patients.     

A case of Oberling's retroperitoneal xanthogranuloma in pilot

A rare case of retroperitoneal mass identified in the pilot, aged 48, is described. There has been noted the complexity of differential diagnosis between the benign and malignant process associated with the given type of tumor which led to some problems when making an expert decision. The tumor was found to be non-removable, however, considering the good clinical state of the pilot, he was permitted of flying activity which he continues to perform successfully more than 10 years. The pilot state remains good which confirms the benign character of the process. The observation is of great interest both clinically and from the flight-surgeon's appraisal standpoint.