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Biomedical subjects

G M Vujanić

Publications and source records attributed to G M Vujanić.

At least 19 recordsLinked to original sources
PubMed2000

Non-Hodgkin's lymphoma of the uterus and CNS.

We report a case of a non-Hodgkin's lymphoma of the uterus and central nervous system in an 8-year-old female. The neurologic signs included blurred vision, neck stiffness, and walking difficulties but no abdominal problems. She deteriorated further, and repeated lumbar punctures revealed the presence of malignant cells in the cerebrospinal fluid. A repeated ultrasound scan of the abdomen demonstrated a markedly enlarged uterus. Biopsy revealed B-cell non-Hodgkin's lymphoma. Treatment according to the Berlin-Frankfurt-Münster protocol was initiated, but she developed hyperventilation syndrome and required mechanical ventilation. Her condition improved after 1 week but then deteriorated again, and despite additional chemotherapy she developed myelosuppression and septicemia with multiresistant Klebsiella pneumoniae and eventually died 13 months after her first admission to the hospital. No clinical or laboratory signs of relapse were evident at the time of death.

Biopsy↗
PubMed2000

Diversion colitis in children: an iatrogenic appendix vermiformis?

AIMS: Diversion colitis (DC) is a localized, relatively benign, iatrogenic condition which occurs in almost 100% of diverted colonic segments in patients who undergo ileostomy/colostomy for various reasons. The aim of this study was to establish histological features of DC in children. METHODS AND RESULTS: Twenty-three cases of DC following colostomy for Hirschsprung's disease in young children were analysed. The distinguishing features included prominent follicular lymphoid hyperplasia (100%), chronic mucosal inflammation (100%), accompanied by a variable degree of acute inflammation (78%) and Paneth cell metaplasia (26%). Less frequent histological findings were as follows: mild goblet cell depletion (22%), foci of cryptitis (13%), crypt abscesses (13%) and mild architectural distortion (22%). A previously unrecognized feature was the presence of mucosal aggregates of eosinophils, found in 43% of cases. A striking similarity between the normal appearance of the vermiform appendix and pathological features in DC was noted and the possible relationship between the two is discussed. CONCLUSIONS: Histological features of DC in children are very similar to those described in adults. They should help to distinguish it from ulcerative colitis and Hirschsprung's-associated enterocolitis in order to prevent inappropriate therapy and follow-up. There are many similarities between DC and the normal appendix vermiformis.

Appendix↗
PubMed1999

New definitions of focal and diffuse anaplasia in Wilms tumor: the International Society of Paediatric Oncology (SIOP) experience.

BACKGROUND: Unlike the original definitions of focal (FA) and diffuse anaplasia (DA) in Wilms tumor (WT), recently redefined FA and DA proved to be of prognostic significance. The aim of the study was to analyze WT from the SIOP file, the majority of which were treated with preoperative chemotherapy, in order to investigate whether chemotherapy influenced the presence of anaplasia, whether the new definitions were applicable to these tumors, and whether they were of prognostic significance. PROCEDURE: The unilateral anaplastic WT of children up to 16 years of age from the SIOP 6 and 9 nephroblastoma trials and studies were first classified according to the original definitions and analyzed. Then they were reclassified and analyzed according to the new definitions. RESULTS: Anaplasia was diagnosed in 86 (5.5%) of 1,554 unilateral WT. The age at diagnosis ranged from 9 to 175 months (median, 63) and more than half of children were over 5 years of age. From 15% to 85% of the tumor mass showed chemotherapy-induced changes. Blastemal anaplasia was seen in 74, stromal in 23, and epithelial in 22 cases. According to the original definitions, FA was diagnosed in 55 (64%) and DA in 31 (36%) cases. In total, 48% children were alive and well, including 53% with FA and 39% with DA (P = 0.23). When reclassified, 39 old FA cases were moved to the new DA group, resulting in 70 (81%) DA and 16 (19%) FA cases. The female-to-male ratio for FA changed from 1.9:1 to 1:1 while remained unchanged for DA. The percentage of FA stage I cases increased from 31% to 44%, while it decreased from 25% to 6% for stage III. For other stages it remained virtually unchanged. The overall 4-year actual survival was 75% for FA and 41% for DA (P = 0.03). CONCLUSIONS: Preoperative chemotherapy did not obliterate or produce anaplasia. The new definitions were applicable to pretreated cases and they were of prognostic significance.

Adolescent↗
PubMed1998

Fibrochondrogenesis in a 17-week fetus: a case expanding the phenotype.

Fibrochondrogenesis is a very rare form of lethal short-limb dwarfism, with 8 cases described since it was first reported in 1978. It is becoming clear that this condition has certain radiological and histological characteristics that distinguish it from other skeletal dysplasias. We herein present a further case of fibrochondrogenesis diagnosed in a fetus of 17 weeks, which is the youngest patient reported so far. In addition, the fetus showed severe micrognathia and a bifid tongue. These are not previously described manifestations, which extend the phenotype of this rare condition.

Abnormalities, Multiple↗
PubMed1998

Triploidy: antenatal sonographic features with post-mortem correlation.

A retrospective study was performed comparing the antenatal ultrasound (US) scan features with post-mortem findings in 20 triploid fetuses surviving beyond the first trimester. US scans showed a range of abnormalities including oligohydramnios (60 per cent of cases), intra-uterine growth retardation (IUGR, 55 per cent), central nervous system (CNS) abnormalities (45 per cent), and placental abnormalities (55 per cent). There were obstetric complications in 30 per cent of cases. Post-mortem examinations confirmed these findings but also demonstrated additional anomalies including facial dysmorphism, limb abnormalities (syndactyly, talips equinovarus and thigh muscle hypertrophy), and anomalies of cardio-vascular, respiratory and genito-urinary system. The placenta was normal in 16 per cent of cases, enlarged with cystic change in 47 per cent, small in 32 per cent and infarcted in 5 per cent of cases. Various factors including oligohydramnios, abnormal fetal position, less sophisticated equipment in mid-1980s and relative inexperience of early observers contributed to the reduced sensitivity of US scans in diagnosis of various defects. However, triploidy should be considered in all cases showing early second-trimester IUGR with or without oligohydramnios, a major CNS abnormality and/or an enlarged placenta with or without changes of partial hydatidiform mole. As triploidy is associated with increased obstetric complications, fetal karyotyping should be offered to confirm the diagnosis so that appropriate obstetric intervention can be instituted.

Abnormalities, Multiple↗
PubMed1998

Pancreatic inflammatory pseudotumour: an uncommon childhood lesion mimicking a malignant tumour.

We describe a rare case of pancreatic inflammatory pseudotumour that clinically presented as a malignant tumour in an 11-year-old boy. We also review all six previously described cases, including three that occurred in children. Complete surgical removal is regarded as the best choice of therapy, and follow-up in our case showed no evidence of disease after 3 years. It is important to be aware of this lesion, since it can be confused with a malignant tumour both radiologically and clinically.

Child↗
PubMed1998

Improving the quality of perinatal and infant necropsy examinations: a follow up study.

AIM: To compare the quality of perinatal and infant necropsy examinations in 1996 with those performed in 1993. METHODS: Cohort analysis, with data from the All Wales Perinatal Survey, of 1027 deaths (540 in 1993; 487 in 1996) of babies between 20 weeks' gestation and one year of age. The quality of the necropsy was assessed by scoring aspects identified as being part of the investigation. RESULTS: Necropsy was performed in 335 cases (62%) in 1993 and in 320 cases (66%) in 1996. The proportion done in a regional centre increased significantly from 39% (131/335) in 1993 to 76% (243/320) in 1996 (p < 0.0001). The quality of necropsy was above the minimum standard in 54% of cases in 1993 (171/314) compared with 93% in 1996 (289/312) (p < 0.0001). Improvement occurred in all categories. For stillbirths, 35% (46/133) were above the minimum standard in 1993 compared with 90% (104/116) in 1996 (p < 0.0001); for cases not classified as sudden unexpected death in infancy (SUDI), the improvement was from 62% in 1993 (40/65) to 97% in 1996 (73/75) (p < 0.0001); and for SUDI cases, the improvement was from 32% in 1993 (10/31) to 91% in 1996 (21/23) (p < 0.0001). The quality of both non-regional and regional necropsies improved. For non-regional cases, the score was above the minimum standard in 28% (51/183) in 1993 compared with 69% (52/75) in 1996 (p < 0.0001); for regional cases it improved from 92% (120/131) in 1993 to 100% (237/237) in 1996 (p < 0.0001). CONCLUSIONS: The quality of perinatal and infant necropsies improved considerably between 1993 and 1996, reflecting better awareness of the importance of good quality examination and an increase in referrals to paediatric centres.

Autopsy↗
PubMed1997

Bilateral femoral agenesis in femoral facial syndrome in a 19-week-old fetus.

Although the cause in most cases is unknown, there is a strong association of the femoral facial syndrome (FFS) with maternal diabetes mellitus. We describe an unusual presentation of FFS in the first pregnancy of a diabetic mother terminated at 19 weeks gestation because of bilateral femoral agenesis diagnosed on ultrasound scan. Autopsy confirmed the absence of the femora and acetabula and the presence of the facial traits of FFS in a female fetus.

Abnormalities, Multiple↗
PubMed1996

Rhabdoid tumour of the kidney: a clinicopathological study of 22 patients from the International Society of Paediatric Oncology (SIOP) nephroblastoma file.

We present 22 (0.9%) cases of rhabdoid tumour of the kidney diagnosed amongst 2392 renal tumours in children. The patients ages ranged from 3 weeks to 94 months (median 7 months) and the female:male ration was 1.2:1. Clinically, they presented with an abdominal mass but four (18%) children also had hypercalcaemia and one (4.5%) developed a brain tumour (primitive neuroectodermal tumour). None of the children presented with stage I disease, five (23%) had stage II, ten (46%) stage III, and five (23%) stage IV disease. Two (9%) patients had bilateral tumours. Histologically, the vast majority (20/22) of the tumours exhibited a classical pattern but other histological patterns were also noted. Immunohistochemical studies performed in 12 cases showed vimentin positivity in all cases, CAM 5.2 in eight, epithelial membrane antigen in six, neuron specific enolase in four, S-100 protein in eight, and desmin in one case. In only 12 of the 22 tumours was there agreement between the reporting pathologist and the panel on a diagnosis of rhabdoid tumour of the kidney. Eight tumours originally diagnosed as rhabdoid tumour of the kidney were found to be other renal tumours and in another ten cases the initial diagnosis was changed by the panel to rhabdoid tumour. Metastases developed in 18 (82%) of the children--in eight they were present at the time of diagnosis and in 10 they developed from 2 weeks to 9 months after initial diagnosis. Metastases were found in the lung, abdomen, lymph nodes, liver, bone and brain. Of the 19 children with adequate follow-up, only two (10.5%) with stage II disease are alive, while 17 (89.5%) died 2 weeks to 20 months after the diagnosis.

Adolescent↗
PubMed1995

B-cell non-Hodgkin's lymphoma presenting as a primary renal tumour in a child.

A 6-year-old boy presented with a unilateral renal tumour thought to be a Wilms' tumour. He had started a treatment but review of the histology and immunohistochemical studies proved the tumour to be a primary renal B-cell lymphoma. This case illustrates the importance of an open-minded approach in the histological examination of small round cell tumours in the kidney and immunohistochemical studies in such cases are strongly advocated.

Antigens, CD↗
PubMed1995

Perinatal and infant postmortem examinations: how well are we doing?

AIM: To investigate the quality of perinatal and infant necropsies and assess the relation between the quality and value of this investigation in different outcome groups. METHODS: Cohort analysis of 540 deaths during 1993 of babies between 20 weeks' gestation and one year of age born to women usually resident in Wales. Cases were identified from the All Wales Perinatal Survey. Each case was assessed to establish whether the necropsy yielded clinically relevant information. The quality of necropsy was assessed by scoring aspects identified as being part of the necropsy. RESULTS: Necropsy was performed in 335 (62%) cases, and the report was available for assessment in 314 cases. The quality of necropsy was below the minimum standard in 46% (143/314) of cases. The highest quality necropsies were carried out on fetal deaths at 20 to 23 weeks' gestation (12% (10/85) below standard), compared with 65% (87/133) below standard on stillbirths and 68% (21/31) on sudden unexpected infant death. Overall, 42% (131/314) of necropsies were performed in a regional paediatric pathology centre including 88% (76/88) of fetal deaths, 23% (31/133) of stillbirths and 30% (29/96) of infant deaths. The quality score for the necropsy performed in a regional centre failed to achieve the minimum acceptable score in 8% (11/131) of cases compared with 72% (132/182) for those done elsewhere. The cause of death was detected by necropsy in 17% (52/314) of cases. The quality of necropsy was significantly higher when the cause of death was revealed than when nothing new was found. CONCLUSIONS: The overall quality of the perinatal and infant necropsy is poor. This is regrettable as valuable information can be revealed frequently by a good quality necropsy. Adherence to Guidelines for Postmortem Reports recently published by the Royal College of Pathologists should improve the situation.

Abortion, Spontaneous↗
PubMed1993

Ultimobranchial body nests in human fetal thyroid: an autopsy, histological, and immunohistochemical study in relation to solid cell nests and mucoepidermoid carcinoma of the thyroid.

The mid portion of the lateral thyroid lobes from 64 fetuses was systematically analysed for the presence of ultimobranchial body nests. The nests showed a prevalence of 1/24 (4.2 per cent) or 13/40 (32.5 per cent) depending on whether a single- or a multi-step sectioning method was employed, respectively, and showed anatomical, morphological, and histochemical features similar to those of ultimobranchial postnatal thyroid solid cell nests. Histochemical studies revealed the presence of mucosubstances in 73 per cent of the cases, calcitonin-immunoreactive cells in 36 per cent, and both carcinoembryonic antigen and high-molecular-weight cytokeratin-immunoreactive epidermoid cells in 85.7 per cent, respectively. These findings indicate that these markers, which are also expressed by solid cell nests of the thyroid, are of value for the detection and tracing of ultimobranchial tissue to earlier stages of development. The findings of this study support the hypothesis that mucoepidermoid carcinomas of the thyroid are of ultimobranchial tissue origin.

Animals↗
PubMed1992

Aggressive inflammatory pseudotumor of the abdomen 9 years after therapy for Wilms tumor. A complication, coincidence, or association?

BACKGROUND: Inflammatory pseudotumor (IPT) is a benign lesion that occurs in various organs and tissues. It is usually sharply demarcated from the surrounding tissue and surgery is considered to be the best treatment. METHODS: This article discusses a 15-year-old boy with an aggressive IPT of the abdomen occurring 9 years after therapy for Wilms tumor. RESULTS: IPT widely involved the esophagus, stomach, and liver, producing severe dysphagia. Histologically, it showed classic features of IPT and, also, areas of metaplastic bone, a new feature recently described in the same lesion of the kidney. It mimicked malignant tumor clinically and led to extensive surgery, but early follow-up has shown no recurrence. CONCLUSION: Although many complications of surgery and chemotherapy are well known, the authors believe that it is unlikely to be the cause of IPT in the case presented. Therefore, the possibility of coincidence or association of Wilms tumor and IPT remains open.

Adolescent↗