Inflammatory myofibroblastic tumors may be a locally aggressive and destructive neoplasm. Tumor recurrence is unusual following complete surgical resection or organ-preserving combined modality therapy.

Metatropic dysplasia is a clinical heterogeneous skeletal dysplasia characterized by short extremities, a short trunk with progressive kyphoscoliosis, and craniofacial abnormalities that include a prominent forehead, midface hypoplasia, and a squared-off jaw. Dominant mutations in the gene encoding TRPV4, a calcium permeable ion channel, were identified all 10 of a series of metatropic dysplasia cases, ranging in severity from mild to perinatal lethal. These data demonstrate that the lethal form of the disorder is dominantly inherited and suggest locus homogeneity in the disease. Electrophysiological studies demonstrated that the mutations activate the channel, indicating that the mechanism of disease may result from increased calcium in chondrocytes. Histological studies in two cases of lethal metatropic dysplasia revealed markedly disrupted endochondral ossification, with reduced numbers of hypertrophic chondrocytes and presence of islands of cartilage within the zone of primary mineralization. These data suggest that altered chondrocyte differentiation in the growth plate leads to the clinical findings in metatropic dysplasia.

Linkage map of the human major histocompatibility complex including the tumor necrosis factor genes (pulsed- ABSTRACTThe tumor necrosis factor (TNF) a and (3 gene pair has been linked in the human major histocompatibility complex to HLA-B, HLA-C, and, tentatively, HLA-E and HLA-A on one side and to the class III complement/steroid, 21-hydroxylase gene cluster on the other by pulsed-field gel electrophoresis. The TNF genes are located 200 kilobases (kb) centromeric of HLA-B and about 350 kb telomeric of the class III cluster. Together with previous data on the linkage and structures of the class II and class III regions, a restriction map of the entire human major histocompatibility complex of about 3500 kb has been prepared.

The terminology and diagnostic criteria presently used by pathologists to report invasive placentation is inconsistent and does not reflect current knowledge of the pathogenesis of the disease or the needs of the clinical care team. A consensus panel was convened to recommend terminology and reporting elements unified across the spectrum of PAS specimens (i.e. delivered placenta, total or partial hysterectomy with or without extrauterine tissues, curetting for retained products of conception). The proposed nomenclature under the umbrella diagnosis of Placenta Accreta Spectrum (PAS) replaces the traditional categorical terminology (placenta accreta, increta, percreta) with a descriptive grading system that parallels the guidelines endorsed by the International Federation of Gynaecology and Obstetrics (FIGO). In addition, the nomenclature for hysterectomy specimens is separated from that for delivered placentas. The goal for each element in the system of nomenclature was to provide diagnostic criteria and guidelines for expected use in clinical practice.

Maternal floor infarction (MFI) is a poorly understood placental lesion reportedly associated with intrauterine growth restriction (IUGR) and recurrence. In this study of MFI and the related placental disorder, massive perivillous fibrin deposition (MFD), semiquantitative histologic criteria for these diagnoses are defined and rates of IUGR and recurrence are assessed. Pathologic slides of 80 placentas diagnosed as MFI or MFD at the Brigham and Women's Hospital (1989-99) were reviewed and reclassified as classic MFI, transmural MFD, borderline MFD, or neither MFI or MFD. The prevalence of IUGR was determined, and placental slides from additional pregnancies were reviewed to evaluate recurrence. Among 25 cases originally diagnosed as MFI, 5 (20%) were reclassified by study criteria as classic MFI, 9 (36%) as transmural or borderline MFD, and 11 (44%) as neither lesion. Among 55 cases originally diagnosed as MFD, 27 (49%) were reclassified as transmural or borderline MFD, 4 (7%) as classic MFI, and 24 (44%) as neither lesion. IUGR was identified in no case with classic MFI, in 31% of cases with transmural or borderline MFD (P = 0.02), and in 11% of cases with neither lesion. Recurrence was documented in 1 of 7 (14%) second- or third-trimester placentas. Possible recurrence was suggested in 3 of 6 (50%) first-trimester spontaneous abortion specimens. Classification of intraplacental fibrin is subjective and problematic; almost half of potential cases of MFI or MFD did not fulfill our study's diagnostic criteria. MFD may be more common and more strongly associated with IUGR than classic MFI. Recurrence of these lesions appears to be infrequent among second- and third-trimester placentas, but may be relatively common in first-trimester spontaneous abortions.