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Biomedical subjects

P Moerman

Publications and source records attributed to P Moerman.

At least 181 records · Page 10Linked to original sources
PubMed1982

Congenital atresia of left coronary ostium.

A two-year-old girl, who presented with congestive heart failure and an extensive anterolateral infarction, was thought to have anomalous origin of the left coronary artery from the pulmonary artery. She improved rapidly and remained symptom free until 13 years of age, when she died suddenly. At necropsy the right coronary artery was found to be normal but in the left aortic sinus a dimple was the only remnant of the left coronary ostium. The proximal segment of the left long term follow-up of a patient with this rare anomaly.

Child, Preschool↗
PubMed1982

Nonimmunologic hydrops fetalis. A study of ten cases.

Ten cases of hydrops fetalis not associated with serologic incompatibility between mother and infant were studied by autopsy. Classic trisomy 21 was present in two, and in six others the postmortem examination revealed major congenital abnormalities (hydrometrocolpos-polydactyly syndrome, achondrogenesis type 2, congenital adenomatoid malformation of the lung, and aortic valvular dysplasia with left ventricular endocardial fibroelastosis). In two instances, anasarca was associated with a congenital tumor: a sacrococcygeal teratoma and an adrenal neuroblastoma. In six fetuses, prenatal diagnosis of hydrops fetalis or severe congenital malformations was established by ultrasonography.

Abnormalities, Multiple↗
PubMed1982

Spectrum of clinical and autopsy findings in trisomy 18 syndrome.

Clinical observations and autopsy findings in 16 cases of full 18 trisomy are presented. These patients were seen during the ten-year period of 1971-1981. In 14 cases, typical clinical features allowed a tentative diagnosis. However, two cases presented with an atypical phenotype: a Potter's syndrome associated with urethral atresia was superimposed, obscuring the more classical trisomy 18 stigmata. This combination is believed to be unique. Cardiovascular defects, varying in type and degree were invariably present. A very high incidence of major gastrointestinal and urogenital abnormalities was also noted. Histological abnormalities giving evidence of organ dysplasia, were observed in the central nervous system, pancreas, kidneys and ovaries.

Abnormalities, Multiple↗
PubMed1980

Cardiovascular malformations in DiGeorge syndrome (congenital absence of hypoplasia of the thymus).

Partial or complete absence of the thymus (DiGeorge syndrome, III-IV pharyngeal pouch syndrome) is often associated with agenesis or hypoplasia of the parathyroid glands and, almost invariably, with cardiovascular malformations. The clinical and pathologcial findings in 10 cases proven at necropsy are presented. All patients presented with cardiac symptoms and signs in the first weeks of life and, with one exception, all died of a cardiac cause. Major cardiovascular malformations were found in all 10 cases. Four had, in association with a ventricular septal defect of the infundibular type, an interrupted aortic arch, which was left-sided in two and right-sided in two other cases. Four patients had truncus arteriosus type I, in two of them associated with a right-sided aortic arch. Two patients with tetralogy of Fallot had a right-sided aortic arch. Only two of the 10 had a normally developed left aortic arch. Aberrant subclavian arteries were found in five cases. From our observations and a survey of the previously published patients it appears that 90 per cent of the necropsy-proven cases of DiGeorge syndrome have cardiovascular malformations and that 95 per cent of these malformations can be classified as aortic arch anomalies, truncus ateriosus, or tetralogy of Fallot.

DiGeorge Syndrome↗
PubMedDate not supplied

The development of the intrahepatic bile ducts in man: a keratin-immunohistochemical study.

The development of the intrahepatic bile ducts in man was studied using an immunohistochemical technique on 56 liver specimens ranging in age from 6 weeks of gestation to 8 months after birth. On paraffin sections, two monoclonal anticytokeratin antibodies (CAM 5.2 and KL-1) that normally stain both hepatocytes and bile duct cells and two polyclonal anticytokeratin antisera that in normal adult liver stain bile ducts only were applied. For immunohistochemical staining of cryostat sections (only available from 14 weeks of gestation on), four monoclonal antibodies specifically directed against individual cytokeratin polypeptides 7,8, 18 and 19 were used. Adult human hepatocytes contain cytokeratin 8 and 18 whereas bile duct cells also express cytokeratin 7 and 19 in addition to cytokeratin 8 and 18. On paraffin sections, primitive hepatocytes were stained with monoclonal antibodies CAM 5.2 and KL-1 from 6 weeks of gestation on. On cryostat sections, they were positive with monoclonal antibodies anticytokeratin 8 and 18 from the earliest time point examined (14 weeks). From 9 weeks of gestation on, portal vein branches were surrounded by a layer of cells showing a stronger positive reaction with monoclonal antibodies CAM 5.2 and KL-1 on paraffin sections and with monoclonal antibodies anti-cytokeratin 8 and 18 on cryostat sections (only available from 14 weeks on). This layer, referred to as the ductal plate, first appeared around large portal vein branches close to the hilum and subsequently around more peripheral branches. The duct plates became duplicated over variably long segments of their perimeter, lumina appeared and tubular structures were formed. The latter gradually became incorporated in the connective tissue surrounding the portal vein, resulting in the appearance of individualized bile ducts. Ductal plate cells were stained by both polyclonal anticytokeratin antisera on paraffin sections. On cryostat sections (available from 14 weeks of gestation on), they were immunoreactive for cytokeratin 19 but negative with the monoclonal antibody directed against cytokeratin 7 until 20 weeks of gestation. From then on, weakly positive staining for cytokeratin 7 was detected, but staining intensity subsequently increased and reached the level observed in adult liver at 1 month after birth. At birth, the smallest branches of the portal vein were still surrounded by a discontinuous ductal plate. We conclude that intrahepatic bile duct cells develop from hepatocytes around branches of the portal vein.(ABSTRACT TRUNCATED AT 400 WORDS)

Antibodies, Monoclonal↗
PubMedDate not supplied

Second trimester prenatal diagnosis of epignathus teratoma in ring X chromosome mosaicism with inactive ring X chromosome.

We report the second trimester prenatal echographic diagnosis of an epignathus teratoma in a female fetus with ring X chromosome mosaicism. The ring X chromosome mosaicism was present in the amniotic cell culture and in the teratoma and the ring X was inactive (X-inactive specific transcript (XIST) locus expressed). Hypoplastic left heart with valvular aortic stenosis and non-immune hydrops were additional findings, and are well-documented in Turner syndrome. The occurrence of epignathus teratoma in Turner syndrome has not been documented sofar.

Amniocentesis↗
PubMedDate not supplied

Endodermal sinus tumor of the vulva: successfully treated with high-dose chemotherapy.

Endodermal sinus tumors (EST) of the lower female genital tract are uncommon malignancies. Most of these tumors are found in the vagina or in the cervix. Only seven cases of EST involving the vulva are reported. We report an eighth case of vulvar EST. The patient recurred and was successfully treated with high-dose chemotherapy and peripheral blood cell transplantation. Because of their location, an early diagnosis of vulvar EST should be possible. Resection of the lesion by using a modified radical vulvectomy followed by cisplatin-based chemotherapy seems the best treatment for these tumors today. Our patient was initially treated with the help of local surgery and adjuvant chemotherapy consisting of three courses of etoposide and cisplatin. Sixteen months after the first diagnosis, pleural and lung metastases were diagnosed. Second-line chemotherapy followed by high-dose chemotherapy with autologous peripheral blood stem cell support was administered after resection of the lung metastases and biopsy of the pleural metastases. There is no evidence of disease 40 months after the diagnosis of the lung and pleural metastases, and 56 months after the primary diagnosis.

Adult↗
PubMedDate not supplied

Immunohistochemical expression of CD10 antigen in uterine adenosarcoma.

Uterine adenosarcoma (UAS) is microscopically characterized by a biphasic growth pattern. By definition, the epithelial component is benign, whereas the stromal component typically has the appearance of a low-grade sarcoma, usually an endometrial stromal sarcoma. CD10 acts by reducing cellular response to peptide hormones and is currently regarded as a specific marker for endometrial stromal tumors. In this international multicenter study, we further explored CD10 immunoreactivity in 30 UASs. We encountered CD10 positivity of the sarcomatous component in 18/20 (90%) of UASs, in five of eight (63%) of UASs with sarcomatous overgrowth as well as in both cases of recurrent UAS. The epithelial component stained negative in all cases. These findings suggest that CD10 can be used to differentiate UAS from cellular leiomyoma, or in case endometrial stromal cells exhibit muscle differentiation. Furthermore, CD10 positivity in recurrent UAS might guide the pathologist toward an endometrial stromal origin.

Adenosarcoma↗
PubMedDate not supplied

Report of an unusual problematic uterine smooth muscle neoplasm, emphasizing the prognostic importance of coagulative tumor cell necrosis.

Follow-up data of the clinical behavior of uterine smooth muscle tumors with low malignant potential are scarce. We present a woman suffering from a uterine smooth muscle cell tumor with increased cellularity, absence of significant atypia, and two to three mitotic figures per 10 HPFs but with minimal focal coagulative tumor cell necrosis (CTCN). These microscopic features are currently accepted to label the lesion as a "smooth muscle neoplasm of low malignant potential, limited experience." After a disease-free survival of 4 years, two retroperitoneal tumors around the iliac vessels were extirpated. Both tumors consisted of smooth muscle cells with mild to moderate atypia and a mitotic index of 5-10 per 10 HPFs but with multifocal and extensive CTCN. These microscopic features were sufficient to establish the diagnosis of leiomyosarcoma. This case adds to the limited experience of the clinical behavior of problematic uterine smooth muscle cell neoplasms and underscores the prognostic importance of CTCN.

Female↗
PubMedDate not supplied

Neoadjuvant chemotherapy followed by radical hysterectomy for invasive cervical cancer diagnosed during pregnancy: report of a case and review of the literature.

Although cervical carcinoma is among the most frequently encountered malignancies during pregnancy, only four cases of neoadjuvant chemotherapy during pregnancy have been reported. A 28-year-old A0P1G2M0 was diagnosed at 15 weeks with stage Ib1 invasive squamous cervical cancer. Because she strongly desired the continuation of this pregnancy, after extensive counseling she was treated with 75 mg/m(2) cisplatin every 10 days starting at 17 weeks. After six cycles, clinically and radiologically stable disease with normalization of the squamous cell carcinoma tumor marker was obtained. An elective cesarean delivery followed by radical hysterectomy and lymphadenectomy was performed at 32 weeks gestation. The pathology report revealed a moderately differentiated squamous cell carcinoma of 3.5 cm, and all 33 lymph nodes were free of disease. Neonatal examination of the baby could not reveal any abnormalities, and this was confirmed at 6 months. The use of neoadjuvant chemotherapy enabled us to continue this pregnancy until the fetus was viable. Cisplatin did not influence the short-term outcome, but only a long-term follow-up will inform us on its safety during pregnancy.

Adult↗
PubMedDate not supplied

Prophylactic salpingo-oophorectomy in 51 women with familial breast-ovarian cancer: importance of fallopian tube dysplasia.

We report our findings on 51 patients with a familial breast and/or ovarian cancer risk who underwent prophylactic bilateral salpingo-oophorectomy (pBSO). Patients undergoing a pBSO between January 1, 1997, and December 31, 2003, at the University Hospitals Leuven were reviewed retrospectively. Out of 51 patients, 37 (72.5%) underwent genetic testing, while the 14 others (27.5%) underwent pBSO on the sole basis of a positive family history only. BRCA1 mutation was found in 35% of the tested group, while 30% were BRCA2 carriers. Mean age at intervention was 45.3 years; 33 patients were premenopausal. In 44 patients (86%), the pBSO was performed laparoscopically, and in seven patients we decided laparotomy because of technical reasons. In four patients hysterectomy was performed. Histopathologically, the presence of epithelial dysplasia was observed in two patients (dysplasia of the fallopian tube in a BRCA2 patient; discrete focal atypia of tubal and ovarian epithelium in a patient with a negative BRCA1/BRCA2 mutation analysis). Peritoneal cytology specimens (n= 28) were all negative. Laparoscopic pBSO is well tolerated. Concomitant hysterectomy should be considered, given the observed fallopian tube dysplasia and the possibility of a tubal remnant after pBSO.

Adult↗
PubMedDate not supplied

Is there a role for neoadjuvant chemotherapy in the treatment of stage IV serous endometrial carcinoma?

Serous endometrial carcinoma (SEC) is an uncommon variant of endometrial carcinoma that is notorious for its aggressive clinical course. Similar to its ovarian counterpart, it has a propensity for early intraabdominal and lymphatic spread. We present two cases of advanced SEC, who were left with no residual tumor after neoadjuvant chemotherapy. After three courses of chemotherapy, both patients underwent interval debulking surgery, resulting in no residual disease. The documentation of chemosensitivity might enable the clinician to select a subpopulation of patients with widespread SEC that might benefit from interval debulking surgery.

Adenocarcinoma, Papillary↗
PubMedDate not supplied

Second case of uterine mesonephric adenocarcinoma.

A second report of an intramural mesonephric adenocarcinoma of the uterus is presented. The histogenesis and clinicopathologic outcome of a surgically staged malignancy add to the insights and experience of this uncommon disorder.

Adenocarcinoma↗
PubMedDate not supplied

Hereditary renal adysplasia: new observations and hypotheses.

Renal agenesis and dysplasia are frequently regarded by pathologists, even pediatric pathologists, as sporadic malformations. We report six fetal autopsy cases of hereditary renal adysplasia (HRA): two pairs of siblings, one case with paternal unilateral renal agenesis, and one case with an autosomal balanced 6p/19q translocation. The main purpose of this paper is to emphasize that nonsyndromal renal agenesis and dysplasia are pathogenetically related and often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. A subsidiary purpose is to present a case of bilateral multicystic dysplasia with a balanced 6p/19q translocation. This observation further supports the assignment of one of the loci for HRA to chromosome 6p.

Abnormalities, Multiple↗