Masao Fukuzawa

A case of von Recklinghausen's disease with bilateral pheochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumors.

A 48-year-old man with neurofibromatosis type 1 presented with chest pain, paroxysmal hypertension, tachycardia, and progressive respiratory insufficiency. Clinical investigation displayed calcified tumors in the anterior mediastinum and pararenal region. Histological examination at autopsy revealed composite tumors consisting of pheochromocytoma and malignant peripheral nerve sheath tumor (MPNST) at two sites: the left adrenal gland and the region surrounding the inferior vena cava, probably corresponding to the right adrenal gland. The MPNST component showed a varied histological appearance, including hyalinized bands with polygonal cells, a cartilaginous and myxoid stroma, a hemangiopericytomatous architecture, and a fibrosarcomatous structure, which suggested osteosarcoma, chondrosarcoma, angiosarcoma, and fibrosarcoma, respectively. In addition, based on the ultrastructural findings, the gastrointestinal tract was involved with mesenchymal tumors showing neurogenic differentiation. These lesions suggest the divergent cellular differentiation of neural crest-derived cells to mesenchymal elements as well as neuroectodermal neoplasms.

Improved detection of medically important fungi by immunoperoxidase staining with polyclonal antibodies

This study was performed to identify pathological fungi of eight species [Aspergillus fumigatus, Candida albicans, Torulopsis (Candida) glabrata, Cryptococcus neoformans, Fusarium anthophilum, Rhizopus oryzae, Sporothrix schenckii and Trichosporon beigelii] in formalin-fixed, paraffin-embedded tissue sections by indirect immunoperoxidase staining. Mature albino rabbits were immunized with formalin-killed organisms. Antibodies were prepared by precipitation. Immunoperoxidase staining was applied to the paraffin-embedded tissue sections of experimentally infected mice and human autopsy and surgical specimens. Although the cell walls of each fungus stained clearly, many cross-reactivities appeared. However, it was possible to obtain specificity for the eight species by absorption and dilution of the antisera.

Ultrastructural study of Cryptococcus neoformans by quick-freezing and deep-etching method

The three-dimensional ultrastructure ofCryptococcus neoformans was studied by quick-freezing and deep-etching (QF-DE) method.C. neoformans, strain CDC551, was cultured on agar. The viable yeast cells (107 cells) were inoculated into each mouse from the tail vein. Three weeks after the inoculation, the brains of the mice were perfused with fixatives, quickly frozen, freeze-fractured, deeply etched and rotary shadowed with platinum and carbon. In addition, the viable cells ofC. neoformans on agar were picked up and quickly frozen, and replica membranes were prepared as described above. The ultrastructure ofC. neoformans was three-dimensionally demonstrated by the QF-DE method. The capsule was composed of fine meshworks of microfibrils (10–13 nm in diameter), which were directly attached to the cell walls. The capsule of the in vivo yeasts (yeast cells in the brain lesion) was thicker than that of the in vitro yeasts (yeast cells on agar culture). At the outer part of the cell wall, a particle-accumulating layer was observed. This layer in vivo was thicker than that in vitro. Occasionally, the yeast cells were ingested by phagocytes in the mouse brain. Although the cytoplasm of such yeast cells was destroyed, the capsular meshworks were well preserved. The ultrastructure of the capsule was the same both in cultured and phagocytized yeasts in the cystic lesions of the brains. This lack of morphological changes of the capsular meshworks suggests that they are resistant to the digestion by phagocytes. This stability of capsular structures may provide one of the important pathogenic factors in cystic lesions byC. neoformans.

Kaposi’s varicelliform eruption of an elderly patient with multiple myeloma

Kaposis varicelliform eruption (KVE) is characterized by disseminated vesiculopustules and erosions due to a herpes virus infection superimposed on a preexisting dermatosis such as atopic dermatitis. However, the occurrence of KVE in patients with multiple myeloma seems to be very rare; to our knowledge, only one such case has been reported. This report documents a second case of KVE in a patient with multiple myeloma.

Effect of high-fat meal intake on the pharmacokinetic profile of ivermectin in Japanese patients with scabies

Ivermectin (IVM) is used as an anthelmintic agent in many countries. To evaluate the effect of high‐fat (HF) meal intake on the pharmacokinetics of IVM, a clinical trial was conducted in Japanese patients with scabies. The patients were administrated Stromectol® tablets in the fasted state, and after 1 week they were also administrated it after a HF meal (fed state). After the administration, IVM concentrations in plasma and the stratum corneum were determined. The geometric mean of fed/fasted ratio of area under IVM concentration‐time curve (AUC) in plasma was 1.25 (90% confidence interval, 1.09–1.43), suggesting the tendency to increased absorption after a HF meal. The fed/fasted ratio of the maximum IVM concentration in the stratum corneum was well correlated with that in plasma. In addition, no serious adverse events were observed during the trial, while a mild increase of aspartate aminotransferase and alanine aminotransferase activity in plasma was observed under the fed state in two patients. The mean AUC of IVM in plasma of those two patients were approximately threefold higher than that of the other patients at that time. On the other hand, the treatment success rate was 76.9% at 7 days after the second administration, which was comparable with the expected level. The present study not only demonstrates that HF meal intake increases the IVM concentration in plasma and the stratum corneum in Japanese patients with scabies, but also suggests the possibility that HF meals increase the risk of hepatic dysfunction by the increased exposure of IVM.

Immunohistochemical, Electron Microscopic, and Immunoelectron Microscopic Features of Plasmacytoma of the Thyroid with Amyloid Deposition

A rare case of primary plasmacytoma of the thyroid with amyloid deposition is reported. The tumor consisted of diffuse proliferation of atypical plasma cells showing IgG lambda-type monoclonal growth. Amyloid deposition with focal giant cell reaction was also observed. Bone marrow aspiration and systemic skeletal radiographic surveys revealed no evidence of multiple myeloma and myelomatosis. Radial arrangement of amyloid bundles was observed ultrastructurally. By immunoelectron microscopic examination, lambda chain was detected in the amyloid fibrils and rough endoplasmic reticulum of the tumor cells. Our findings suggest the following: amyloid fibrils originate from monoclonal light chains produced by tumor cells, and histiocytes contribute to amyloid deposition as well as to giant cell reaction in extramedullary plasmacytoma.

Systemic Plasmacytosis with Aseptic Bone Necrosis and High Fever

To the Editor: A Japanese woman, diagnosed with systemic plasmacytosis at the age of 40 and previously reported by Uhara et al. (1), incurred lymphoid interstitial pneumonia at the age of 46 without any symptoms. After the diagnosis, her skin lesions, serum IgG (3,500 mg/dl; normal 800–2,000 mg/dl), and interleukin (IL)-6 level (8.0 pg/ml; normal <4 pg/ml) remained stable until November of 1999, when the patient’s IgG level increased to 4,767 mg/dl without any symptoms. Two weeks later, she was admitted to our hospital with complaints of chills and bilateral knee pain. Her body temperature was 39.3°C, but physical examination could not detect any abnormal findings in her legs. Laboratory data demonstrated increases in CRP (9.95 mg/dl), endotoxin (60.9 pg/ml), and beta-D-glucan (13.6 pg/ml), and her IL-6 had also increased to 351 pg/ml. These findings strongly suggested that an unknown infectious episode had had an important effect on her condition. However, cultures from blood, urine, and tonsils detected no causative organisms, and the chest X-ray indicated lymphoid interstitial pneumonia but without any sign of progression. Bone marrow aspiration and biopsy, abdominal computed tomography (CT), brain CT, and ophthalmological, endocrinological and gynecological examinations showed no abnormalities. Re-biopsy of the skin lesion showed the typical findings of cutaneous plasmacytosis (Fig. 1) and 67 Gallium-citrate imaging indicated hot spots in the knee joints. T1-weighted images of MRI demonstrated multiple areas of high signal intensity with a low signal in the outer rims of the tibia and femur (Fig. 2). The lesions showed a low intensity signal with a high signal in the outer rim by T2 and short tau inversion recovery images. These findings are known as the double-line sign and are specific for aseptic bone necrosis (ABN) (2). MRI images of plasmacytic neoplasms may show tumor foci as areas of low signal intensity on T1 images and of high signal intensity on T2 (3). Because other bone lesions without the double-line sign show the opposite pattern (Fig. 2), we could rule out the possibility of plasma cell neoplasms in the case of our patient (3). The final diagnosis of ASB was reached on the basis of MRI findThe Journal of Dermatology Vol. 31: 696–698, 2004

Apocrine Adenocarcinma of the Left Axilla; Report of a Case

We experienced a case of apocrine adenocarcinoma of the left axilla. The patient was an 83-year-old man, who had a reddish indurated plaque, 26×26×6mm in size and partly ulcerated, on his left axilla. Biopsy of the lesion showed the features of adenocarcinoma. Because no other neoplasm was found in other organs of the body, we considered the adenocarcinoma as a primary lesion. The patient underwent local resection of the lesion along with lymphnode biopsy under local anesthesia, as he had suffered from cerebral infarction one month before the surgery. Histopathologic examination of the excised lesion revealed features of moderately differentiated adenocarcinoma showing decapitation secretions in glandular structures. The stroma showed marked fibrosis. The diagnosis of apocrine adenocarcinoma was established. The biopsied lymph node revealed metastasis to the node. After the surgery, adjuvant radiotherapy was given to the left axilla. The patient is currently well 24 months after the radiotherapy. [Skin Cancer (Japan) 2003; 18: 165-168]