Intrasellar plasmacytoma: an unusual presentation of multiple myeloma.

INTRODUCTION: Plasmacytomas are unusual causes of a sellar mass. Occasionally, they can be misdiagnosed as a nonfunctioning adenoma because of radiological and clinical similarities. LITERATURE REVIEW: We reviewed the pertinent literature and discuss here in the light of an illustrative case of our own. DISCUSSION: A 70-year-old woman presented with a recurrent hypophysial mass. Initial diagnosis of a nonfunctioning pituitary adenoma was later overruled by a repeat biopsy, which showed a plasmacytoma. The tumor stained positively for CD138 and kappa light chain. Further studies confirmed the diagnosis of multiple myeloma. The patient was successfully treated with radiotherapy followed by systemic chemotherapy. Because they have different therapeutic implications, extramedullary plasmacytomas involving pituitary gland should be considered in the differential diagnosis of a nonfunctioning pituitary mass.

Retrorectal hemangiopericytoma: a case report.

Hemangiopericytoma is a rare, vascular soft tissue tumour originating from the pericytes, the contractile cells that surround capillaries. A case of retrorectal hemangiopericytoma in a 49-year-old woman is reported. With a posterior sagittal approach, the tumour was completely removed. Pathological examination, including immunohistological stains, was consistent with a hemangiopericytoma. A step-by-step approach to a retrorectal tumour is exemplified, and a very rare pathological entity is added to the spectrum of retrorectal masses.

GATA3 expression and its relationship with clinicopathological parameters in invasive breast carcinomas.

GATA3, as a transcription factor, is associated with estrogen receptor (ER) expression and necessary for luminal cell differentiation in mammary glands. Association of GATA3 expression with clinicopathological parameters, molecular subtypes of tumors, disease free survival (DFS) and overall survival (OS) for breast carcinoma patients were evaluated in this study. We immunohistochemically stained GATA3, CK5/6, EGFR, CK14 and vimentin on tissue microarray blocks of 457 invasive breast carcinomas. Tumors are sub-classified as luminal A, luminal B, HER2 expressing, basal-like and null type according to their hormonal status with cerbB2, CK 5/6 and EGFR expressions. Follow-up data for 254 cases were obtained. 215/457 (47%) tumors were GATA3 positive. GATA3 expression was inversely correlated with mitotic count (p<0.0001), nuclear grade (p=0.001), histological grade (p=0.001), tumor necrosis (p=0.001), stromal lymphocytic response (p<0.01), nipple invasion (p=0.01), metastasis (p=0.03), vimentin (p=0.0003), EGFR (p=0.015) and CK14 (p=0.001) expressions; and directly associated with ER (p<0.0001) and progesterone receptor (PR) (p<0.0001) expressions. Luminal A carcinomas had the highest frequency for GATA-3 (140/245), however basal-like carcinomas had the lowest (1/42) (p<0.0001). None of the medullary and metaplastic carcinomas expressed GATA3. GATA3 was associated with good DFS and OS (p=0.001 and p=0.0009) and was an independent prognostic factor for OS. GATA3 expression, regardless of the subtype, may have a prognostic significance for breast carcinomas through its ability to promote the differentiation of luminal progenitor cells.

Unusually high frequency of a 69-bp deletion within the carboxy terminus of the LMP-1 oncogene of Epstein-Barr virus detected in Burkitt's lymphoma of Turkish children.

Burkitt's lymphoma (BL) in Turkish children is commonly associated with Epstein-Barr virus (EBV) infection. The C-terminus of the latent membrane protein 1 (LMP-1) of EBV is essential for transformation and the 30-bp deletion detected in this region has been implicated to be associated with a more aggressive malignant phenotype. To understand the molecular mechanisms underlying EBV pathogenesis in BL of Turkish children, we analyzed 30-bp deletion and 33-bp variable repeat regions of the LMP-1 gene from paraffin-embedded tumor tissues of 30 BL patients (mean age 5.9 years). Primer pairs spanning the 30-bp deletion and 33-bp repeat regions were designed for amplification by polymerase chain reaction (PCR). The PCR-amplified products were analyzed by gel electrophoresis, Southern blot hybridization, and DNA sequencing. Twenty-eight (93%) of 30 BL biopsy samples were EBV positive as determined by PCR. Variable copy numbers (ranging from 4.5 to 7) of the 33-bp repeat of LMP-1 gene were detected in these EBV-containing tumor samples. To determine the frequency of the 30-bp deletion of the LMP-1 gene, we sequenced the amplimers encompassing this region. Analyses of DNA sequence of 28 Turkish BLs have disclosed four patterns: the first (32% (9/28)) is identical to B95-8 with no deletion, the second (11% (3/28)) is identical to Asian NPC CAO strain with 30-bp deletion, the third (46% (13/28)) is prevalent in Turkish BLs with a longer deletion (69 bp), and the fourth (11% (3/28)) consists of a mixture of 30-bp and 69-bp deletion. The occurrence of high frequency of the 69-bp deletion appears to have no correlation with the disease site. Mutations found in the CAO strain were also detected in the Turkish BL clustering at the amino acids 322, 334, 338 and 342; whereas mutations specific for Turkish BL were clustered at amino acids 326, 352 and 361. To assess the EBV genotype with the changes in C-terminus of LMP-1 gene, we performed genotyping by PCR to differentiate type A and B strain. All 28 patients were infected by type A EBV. Such a high frequency of the larger size (69 bp) deletion has never been reported. Ascertaining the role of this deletion in BL pathogenesis will require further study.

The protective effects of Iloprost and thromboxane synthetase inhibitor, UK 38485, against glycerol--induced acute renal failure in rats.

Tissue protective activities of Iloprost, a stable analogue of PGI2, and of UK 38485, an inhibitor of thromboxane synthetase, were investigated in rats, in which acute renal failure was elicited by the injection of glycerol. The effects of these compounds on PGE2- and LTC4-like activities in the kidney tissue were also studied. Glycerol injection caused acute kidney damage as evidenced by light microscopic examination and abundant hematuria. Glycerol injection also caused an increase in tissue PGE2- and LTC4-like activities. Although both metabolites were increased, the ratio of PGE2/LTC4 was found to be decreased when compared with the control value. Both Iloprost and UK 38485 partially prevented tissue damage due to glycerol and caused an increase in the ratio of PGE2/LTC4. The preventive effects of the drugs were more pronounced when both drugs were used in combination. The participation of arachidonic acid metabolites in the mechanism of the production of kidney damage due to glycerol and possible preventive effects of the compounds are discussed.

Burkitt's lymphoma in Turkish children: clinical, viral [EBV] and molecular studies.

Eighty-one Turkish children with Burkitt's lymphoma (BL) were observed during a period of 24 years (1968-1992). The diagnosis was established histologically according to WHO criteria. BL represented 48.5% of NHL in this series. The median age of patients was 5 years with a sex (M/F) ratio of 2.3/1. The most common primary site of tumor involvement at initial presentation was the abdomen (70.4%), which was followed by facial tumors, in particular the jaw and orbit (45.7%). The majority of the patients (84.0%) were in advanced stages (C and D) at initial diagnosis. Facial tumors observed in Turkish children with BL were more similar to African Burkitt's lymphoma than American or European cases. High titers of antibodies against VCA and EA of EBV were also observed in 32 recent cases of BL. Preliminary molecular and immunologic studies revealed EBV-DNA (type I) and T cell deficiency. The clinical presentation, median age, and association with EBV revealed that BL appears to be inbetween African and non-African types in Turkish children. This will be further elucidated in the future by direct examination of tumor cells for EBV and investigation of the molecular characteristics in these cases.

Malakoplakia of the skin. Ultrastructure and quantitative x-ray microanalysis of Michaelis-Gutmann bodies.

The etiologic agent in a case of malakoplakia of the skin was identified as Staphylococcus aureus from culture of the lesion and from ultrastructural observations. Ultrastructurally, electron-dense inclusions were observed in addition to membranous whorls and Michaelis-Gutmann (MG) bodies. These were labeled "precalcification formations" since they possessed an organic crystalline structure. These formations may act as nucleation centers for further incorporation of organic and inorganic material. Deposition of fine crystalline material in the outer region may occur as the structure becomes saturated with inorganic elements, thus completing formation of the MG body. Quantitative x-ray microanalysis of MG bodies demonstrated the presence of phosphorus, calcium, and iron, with average concentrations of 2.1%, 2.6%, and 0.7% by weight, respectively.

Hungry bone syndrome in a child following parathyroid surgery.

The rare hungry bone syndrome was encountered in a 15-year-old child after the removal of a parathyroid adenoma. Contrary to the hypocalcemias caused by the removal of all parathyroid glands or transient ischemia after parathyroid surgery, in which the serum inorganic phosphorus level is usually normal, both serum calcium and inorganic phosphorus levels are decreased in hungry bone syndrome in the early postoperative period. Vigorous calcium supplementation and vitamin D are required for prolonged periods.

Mitral valve and left ventricular thrombi in an infant with acquired protein C deficiency. A case report.

The authors present an infant with left ventricular and mitral valve thrombi diagnosed by cross-sectional echocardiography. Thrombosis was due to acquired transient protein C deficiency, which was caused by impaired liver function due to sepsis. Because the thombi were very large and mobile, urgent surgery was performed. Eight weeks later, the patient's protein C level returned to normal ranges. The authors suggest that in all cases with intracardiac thrombosis, protein C deficiency should be investigated.

Aggressive Kaposi's sarcoma in children: a case report.

A three-year-old-boy with generalized Kaposi's sarcoma (KS) is presented. The child died of progressive pulmonary insufficiency on the eighteenth day of the course of his illness, the tenth hospital day. On postmortem examination diffuse KS infiltration was observed in the respiratory and gastrointestinal tracts, lymph nodes, liver, spleen and thymus. The patient was considered to be a case of KS unrelated to AIDS because of his negative HTLV-III antibody and epidemiologic characteristics, and therefore was believed to have primary aggressive KS.

An unusual clinical presentation of non-Burkitt's lymphoma in a child. Case report.

A case of non-Burkitt's lymphoma (NBL) in a 31 months old female child is presented. Her kidneys were bilaterally enlarged. Although no abdominal mass was palpable, a sudden and unexpected bowel perforation has occurred. The diagnosis was established by autopsy studies. Because of its extremely rapid progression and in order to minimize the risk of life-threatening complications, children with non-Hodgkin's lymphoma (NHL) should be quickly and extensively evaluated.

The significance of histopathological changes of the normal tunica albuginea in Peyronie's disease.

Five patients with Peyronie's disease were operated and the tissue specimens from the plaque involving the tunica albuginea and from the tunica of the unaffected contralateral side were obtained. The specimens were then subjected to histopathological examination and the findings were correlated with those of men proved not to have Peyronie's disease but operated for other reasons. In the specimen of plaque, the changes varied from collagen destruction to cartilaginous and osseous metaplasia. However, in the tunica of the contralateral side the changes such as disregularity of collagen bundles extending even to widespread focal areas, and appearance of pale, rough, eosinophilic collagen degeneration, instead of its characteristic bright birefringence, and focal mucinous degeneration were noted. Interestingly, these alterations were not observed in the specimens of normal tunica albuginea in the control group. But the existence of minimal alterations, the regularity in the apparently normal contralateral tunica albuginea specimens in Peyronie's disease implied that there was an active turnover in collagen production, and the possible factor causing collagen degeneration and so plaque formation was probably the microtraumatization of the tunica albuginea.

Rete testis adenocarcinoma recurring in the inguinal lymph nodes. A case report.

Adenocarcinoma of the rete testis is a rare neoplasm with 41 reported cases in the literature till 1994. In most of the reported cases, the neoplasm presents as a scrotal mass with diffuse enlargement. The aetiology is unknown and the clinical course of the tumour is not very well defined. In six of the reported cases metastatic spread of the tumour to inguinal lymph nodes was demonstrated in the follow-up. We report herein a distinctive case of rete testis adenocarcinoma presenting as an isolated inguinal recurrence one year after radical orchiectomy.

Non-specific inflammation and bone marrow depletion due to intramedullary porous hydroxyapatite application.

Calcium hydroxyapatite (HA) ceramics are widely used in orthopaedic surgery and are known to be biocompatible, biodegradable, and osteoinductive. Recent studies of HA have identified early stage inflammatory reactions and third-body wear following hip replacement surgery. The aim of this study was to investigate the early stage inflammatory response toward HA by means of conventional radiography, histopathology, and scanning electron microscopy (SEM). HA was implanted into the medullary area of the tibiae of rabbits and followed from one week to six months. Radiography revealed local osteoporosis on Week 6 and Month 6 in some, but not, all rabbits. SEM presented favorable bonding between the implant and the cortical bone. Eosinophilic deposition, non-specific local inflammation, and bone marrow depletion were the histopathological findings. Multinuclear cells were identified in the inflammation area. Although bone ingrowth into the pores of the HA was demonstrated, there is no doubt that HA may cause an early inflammatory reaction in the bone marrow. This inflammatory reaction is followed by bone marrow depletion and may be the early sign and the cause of HA-coated implant failure.

Focal dermal hypoplasia (Goltz syndrome) with horseshoe kidney abnormality.

A 10-year-old girl had focal dermal hypoplasia (Goltz syndrome). She showed the characteristic skin manifestations, mental and physical underdevelopment, and facial, dental, skeletal, ophthalmologic, and urinary abnormalities. In addition, she had a horseshoe kidney abnormality.

Keutel syndrome: a report of four cases.

We report four new cases of Keutel syndrome. Clinical manifestation included abnormal cartilage ossification, multiple peripheral pulmonary stenosis (PPS), brachytelephalangism, subnormal IQ, repeated respiratory infections, otitis media and hearing loss. All four children have a typical facial appearance and are of consanguineous parents. Father and the fifth offspring exhibit a normal phenotype; the mother has pulmonary stenosis. This observation confirms Keutel syndrome as a distinct autosomal recessive syndrome.

Leiomyosarcoma of the great saphenous vein.

Primary malignant tumours of the great saphenous vein are extremely rare with only 15 cases reported in the English literature since 1919. We report a patient who survived 5.5 years after treatment with surgery and chemotherapy before dying from lung metastases.