Prolactin-cell adenoma with signet-ring cells: a case report.

OBJECTIVE: Signet-ring cell changes in the pituitary adenomas are extremely rare. To date, there have been only two reports documenting signet-ring cells in pituitary adenomas, one in a growth-hormone cell adenoma and the other in a nullcell adenoma. This report describes, for the first time, signet-ring cells in a prolactincell adenoma. CASE HISTORY: The patient is a 46-year-old male who presented with severe headache and acute on chronic visual loss. Radiographic studies demonstrated a large cystic pituitary lesion with evidence of pituitary apoplexy. Laboratory values were consistent with a prolactin-cell adenoma. The patient underwent transsphenoidal resection of the prolactin-cell adenoma with significant post-operative improvement. RESULTS: The tumor was composed of sheets of monomorphic round cells with conspicuous nuclei and granular cytoplasm, consistent with pituitary adenoma. Many cells had eccentric, often crescentic-shaped nuclei, imparting a signet-ring appearance and immunostaining was positive for prolactin, denoting an atypical prolactin-cell adenoma. The MIB-1 labeling index was slightly elevated. Electron microscopy demonstrated the presence of vacuolated areas in the cytoplasm that were not membrane bound and did not have specific inclusions. DISCUSSION: This case augments the literature on pituitary adenomas with signet-ring cells. The clinical significance of signet-ring cells in pituitary adenomas is unknown. Accumulation of clinical cases, together with the advances in molecular techniques and experimental models, may yield further insight.

Primary atony of the lower uterine segment as a distinct cause of early postpartum haemorrhage: a case series and management recommendations.

Primary atony of the lower uterine segment appears to be a distinct cause of postpartum haemorrhage. We report a case series of women with postpartum haemorrhage where ultrasound and clinical findings revealed a well contracted fundus and upper uterine segment and a ballooned out lower uterine segment, a condition we have called primary atony of the lower uterine segment. We hope that this case series will lead to increased recognition of this condition, stimulate others to report their experience and lead to additional studies to better characterise this entity, and develop more effective therapies.

Prenatal diagnosis of portal vein thrombosis by ultrasound.

Neonatal portal vein thrombosis (PVT) is a rare condition seen in the setting of thrombophilia or after umbilical vein catheterization. We report a case of fetal PVT with abnormal antenatal ultrasound findings at 27 weeks of gestation. This presented initially as dilation of the intrahepatic umbilical vein. To our knowledge, this is the first reported case evaluated prenatally. Color Doppler ultrasound was valuable in detecting constriction of the vessel with a high-velocity jet seen past the obstruction site. A discussion of this condition and differential diagnosis is presented.

Development of an equipment and supply list for emergency medical services delivery at an annual air show.

INTRODUCTION: Reports of medical care at mass gatherings reflect variability in mission and delivery models. Equipment recommendations are similarly varied. Thoughtful pre-planning and experience-based analysis are the best mechanisms for defining general and specific equipment recommendations. OBJECTIVE: This report presents a suggested supply and equipment list developed over a six-year period of medical coverage at an air show, with an emphasis on the usage and cost of expendable supplies. METHODS: The authors were involved in the planning for and execution of emergency medical care for a large, local, military air show on an annual basis, including provision of expendable medical supplies. A list of such supplies was developed over the initial two to three years, formalized and refined over the subsequent two years, and analyzed in the final, highest patient volume year of coverage. Detailed usage and cost was tracked over the final year for expendable supplies. RESULTS: The results of this analysis indicate that comprehensive emergency medical care from first aid to mass casualty care can be offered at reasonable equipment and supply costs, if existing equipment resources can be supplemented by expendable supplies from a pre-determined list. Given the need for large quantities of supplies for a mass casualty contingency and the low likelihood of occurrence, a loan arrangement with a supplier, with return of unused supplies, is particularly convenient and economical. The approach used in this study should be appreciable in other similar settings. In concurrent scheduled events, the iterative process described can lead to greater specificity of needs for expendable supplies.

Erythema nodosum after smoke inhalation-induced bronchiolitis obliterans organizing pneumonia.

Bronchiolitis obliterans organizing pneumonia and erythema nodosum are immunologic diseases that have not been reported to occur together. We report the case of a lady who developed bronchiolitis obliterans organizing pneumonia and erythema nodosum simultaneously, several weeks after smoke inhalation in a house fire.

The construction and utility of an amniocentesis trainer.

OBJECTIVE: Our goal was to design an inexpensive amniocentesis trainer for instruction and practice with ultrasonography-assisted needle guidance and the freehand technique. STUDY DESIGN: The amniocentesis trainer was constructed from a commercially available 5.3-L storage box (No. 5805; Rubbermaid Incorporated, Wooster, Ohio). Sonodense sperical targets 2.3 cm in diameter were taped to the bottom, and the box was filled with a gelatin mixture. The box was covered with a rubber membrane from a pelviscopy trainer (United States Surgical Corp, Norwalk, Conn). After the gelatin mixture set overnight at 40 degreesF, the amniocentesis trainer was ready for use. RESULTS: When a needle is introduced through the membrane into the gelatin, the ultrasonographic image is a reasonable simulation of an amniocentesis. The trainer improved the ability of an operator to perform a freehand amniocentesis, orient the ultrasound transducer, follow a needle with continuous ultrasonographic guidance, and hit a 2-cm target. The gelatin also allows for evaluation of improvement between amniocentesis attempts because a faint image of the needle track from prior attempts remains visible. Once basic amniocentesis skills are mastered, the trainer is easily modifiable so that obstacles can be added that could simulate umbilical cord and fetal extremities. CONCLUSIONS: We were able to construct an inexpensive amniocentesis trainer that facilitates instruction in the freehand amniocentesis technique. This trainer enables an operator to master the skills necessary to perform the technique of freehand ultrasonography-guided amniocentesis under direct ultrasonographic visualization without putting a patient through the discomfort of participating in the learning curve.

CA-125 and carcinoembryonic antigen assay vs. cytodiagnostic experience in the classification of benign ovarian cysts.

OBJECTIVE: To compare the relative strengths of two factors involved in making an accurate differentiation between functional and epithelial ovarian cysts, along with their combination: (1) the cytologist's level of experience in interpreting ovarian cytology, (2) the use of the tumor markers carcinoembryonic antigen (CEA) and CA-125 in cyst fluid, and (3) a combination of (1) and (2). STUDY DESIGN: Papanicolaou-stained sediments from fluid aspirated from 31 resected ovarian cysts (6 functional and 25 epithelial) were blindly and independently evaluated by five pathologists with varying experience in ovarian cytology. Cyst fluid supernatant was used for CEA, enzyme-linked immunosorbent assay, and CA-125 radioimmunoassay; CEA levels > 5 ng/mL or CA-125 > 5,000 U/mL were considered elevated. Cysts were categorized cytologically and histologically as functional or epithelial and by tumor markers as "neither elevated" or "either or both elevated" (EBE). RESULTS: The agreement of histologic diagnosis with each pathologist's cytologic diagnosis ranged from 53% to 84% (53%, 71%, 83%, 82%, 84%), corresponding to increasing level of experience. The percentage of agreement with EBE was 77%, whereas combined experienced pathologist's diagnosis and EBE was 87%. Kappa equaled .45 for experienced cytopathologist's diagnosis or EBE alone. Kappa equaled .53 when the pathologist or EBE diagnosed an epithelial cyst, indicating results unlikely to occur by chance. CONCLUSION: The distinction of functional from epithelial ovarian cysts is best achieved by combining measurement of the tumor markers CEA and CA-125 with a high level of cytopathology experience.

Characteristics of successful claims for payment by the Florida Neurologic Injury Compensation Association Fund.

OBJECTIVES: Our purpose was to examine the obstetric characteristics of claims paid by the State of Florida after the birth of a neurologically impaired child. STUDY DESIGN: The Florida Birth Related Neurological Injury Compensation plan is a no-fault alternative to litigation for compensation after a catastrophic neurologic birth injury. The plan has specific criteria for inclusion. We retrospectively analyzed claims for compensation that were accepted and paid (n = 64) after a birth-related neurologic injury. Simple description statistics were compiled for the relative frequencies of various obstetric correlates found in successful claims for payment. RESULTS: Seventy percent of infants (45) were delivered by cesarean section and 15 of 19 vaginal deliveries (79%) were operative (forceps or vacuum), yielding a 94% operative delivery rate. A persistent nonreassuring fetal heart rate tracing was seen before delivery in all cases. The 5-minute Apgar score was < or = 6 in 91% of deliveries and the 10-minute Apgar score was < 6 in 86% of deliveries. When first examined in the labor and delivery suite, 17 women had a nonreassuring fetal heart rate, and a nonreassuring tracing developed in labor in 47. Nine attempts at vaginal birth after a cesarean section led to a uterine rupture. Seven of these deliveries were either inductions or augmentations against an unfavorable cervix. Forty-five percent (27) of deliveries were associated with meconium-stained amniotic fluid, including 17 infants with meconium aspiration syndrome. There were three shoulder dystocias and four infants with group B streptococcal sepsis. In eight cases (12.5%), there appeared to be a breach of the published standard of care, which contributed to the poor outcome. CONCLUSION: Most of these cases should not have been eligible for compensation in a traditional tort-based system because the applicable standard of care was not breached. Meeting the published standard for perinatal care failed to prevent these devastating neurologic injuries. Obviously, not all intrapartum injuries can be prevented; however, if we are to prevent similar injuries in the future, we will need to examine the clinical management in these or similar case for clues to develop novel strategies to respond to intrapartum emergencies. An unexpected finding was the frequency of catastrophic birth injuries after an attempted vaginal birth after cesarean section with the predominance of these deliveries associated with oxytocin stimulation against an unripe cervix. It is apparent that the push to lower cesarean section rates is not without some risk.

Loss of DCC expression and glioma progression.

The deleted in colorectal cancer (DCC) gene, a candidate tumor suppressor gene on chromosome 18q21, encodes a neural cell adhesion molecule family protein that is most highly expressed in the nervous system. To address the hypothesis that DCC may play a role in glioma development and/or progression, we examined DCC expression by immunohistochemistry in 57 resected human astrocytic tumors. Overall, low-grade astrocytomas were predominantly DCC positive (15 of 16, or 94%), whereas high-grade tumors significantly less often expressed the DCC protein (27 of 41, or 66%; P = 0.03). We were able to directly assess the relationship between DCC expression and tumor progression in 15 patients who initially presented with a low-grade astrocytoma and subsequently recurred with a glioblastoma. Within this panel of paired lesions from the same patient, 14 of 15 (93%) low-grade tumors expressed the DCC protein, whereas only 7 of 15 (47%) corresponding glioblastomas were DCC positive. We also observed that secondary glioblastomas resulting from malignant progression of low-grade astrocytomas were more often DCC negative (8 of 15, or 53%) compared with primary or de novo glioblastomas (6 of 26, or 23%; P = 0.05). These findings implicate DCC inactivation in glioma progression and also demonstrate that DCC expression is preferentially, but not exclusively, lost in the genetic pathway to secondary glioblastoma multiforme.

Persistent patent urachus with allantoic cyst: a case report.

Patent urachus results when there is a persistence of an allantois remnant which normally undergoes atresia during embryological development. It can lead to an abdominal wall defect similar in appearance on ultrasound to an omphalocele. A 34-year-old primigravida presented at 19 weeks' gestation for evaluation of a cystic mass arising at the umbilical cord insertion. The initial impression of the referring physician was an omphalocele. The mass arose from the abdominal wall and the umbilical cord inserted into the membranous covering of the mass, which appeared to be fluid-filled and separate from but contiguous with the urinary bladder. Serial sonography followed the progression of the abdominal wall mass. At term, the patient underwent primary Cesarean section with delivery of a 4494-g male infant. The infant underwent repair and closure of the patent urachus and plastic reconstruction of the abdominal wall. When the urachus remains patent, it can lead to a urinary fistula which mimics the ultrasound appearance of an omphalocele. However, patent urachus is associated with a much lower rate of abnormalities than omphalocele, yielding a better fetal prognosis.

Torulopsis pneumonia. A case report and review of the literature.

Torulopsis glabrata is a rare cause of pneumonia in immunocompromised patients. We herein describe the case of an elderly man who presented with fulminant Torulopsis pneumonia and septic shock leading to death. We then review the literature, describe the clinical syndrome, and delineate an approach to diagnosis and treatment of Torulopsis pneumonia.

Diagnostic use of immunohistochemistry in neuropathology.

Immunohistochemistry has revolutionized both the research and diagnostic endeavors of anatomic and surgical pathologists, including neuropathologists. These methods, which use the high specificity of antibody recognition of target antigens, have largely replaced the more capricious, less sensitive, and less specific panoply of metallic impregnations and other histochemical methods that once were characteristics of neuropathology. With immunostaining, specific infectious agents, from ordinary bacteria to spirochetes, fungi, parasites, and especially viruses, can be identified as the causative agents of central nervous system diseases. Understanding of the pathogenesis and establishment of correct diagnoses of neurodegenerative disorders are both improved, and more precise and reliable diagnosis of central nervous system tumors has become a matter of immunophenotypic characterization using panels of relevant antibodies to supplement the recognition of standard histologic patterns. Newer trends in neuropathologic immunohistochemistry point to a better understanding of the molecular pathology of degenerative disorders and of tumors as specifically mutated oncogenetic or neurotoxic antigens are localized and identified in diseased brain and spinal cord tissues.

Primary neuroectodermal tumors of the ovary. A report of 25 cases.

Twenty-five primary ovarian neuroectodermal tumors occurred in females from 6 to 69 (average, 23) years of age; they had the usual presenting symptoms of abdominal swelling or pain. The tumors, which varied from cystic to solid, ranged from 4 to 20 cm (average, 14 cm) in diameter. Microscopic examination revealed three histologic categories--differentiated, primitive, and anaplastic--with the tumors in the first group having a better prognosis than those in the other two groups. Five of the six differentiated gliomas were pure ependymomas, and one was an ependymoma with an astrocytoma component; none contained teratomatous elements. Two patients with stage I tumors were alive 4 and 5 years postoperatively. The one patient with stage IIA tumor was free of disease at 3 years; one of the two patients with a stage III tumor died of tumor after 5 years, and one had two recurrences but was alive and well at 5 years. Twelve tumors were primitive, resembling medulloepithelioma, ependymoblastoma, neuroblastoma or medulloblastoma. Seven tumors had teratomatous foci of other types, including three dermoid cysts. Three patients with stage I tumors were alive at 7 months, 3 years, and 9 years postoperatively; six of seven patients with stage III tumors died of tumor 2 to 20 months postoperatively, and one was alive with disease at 1 year. Seven tumors were anaplastic, resembling glioblastoma. All contained foci of squamous epithelium. One patient with stage IA tumor died of tumor at 2 years, but two were free of tumor after 3 and 4 years. One patient with a stage IIA tumor died of disease after 5 years; another was alive with tumor at 1 year. One patient with a stage III tumor died after 4 months. The differential diagnosis of neuroectodermal tumors of the ovary includes many primary and metastatic ovarian neoplasms of diverse types, and distinction among them is important. Neuroectodermal tumors should be considered when examining unusual ovarian tumors, particularly if the patient is young.

Demyelinating disease versus tumor in surgical neuropathology. Clues to a correct pathological diagnosis.

Clinical presentations as well as radiological and histopathological findings in biopsies from patients with multiple sclerosis (MS) or other demyelinating disorders of the central nervous system are sometimes misleading, resulting in an erroneous diagnosis of brain or spinal cord tumor. We report 17 patients who presented with symptoms mimicking those of brain (14 cases) or spinal cord (three cases) tumors. Computerized tomography or magnetic resonance imaging studies or both were interpreted as consistent with a tumor in each case. All patients underwent surgery, and all 17 pathological specimens were eventually diagnosed as showing demyelinating disease, usually consistent with MS. In each case we examined a variety of histological features and immunohistochemical studies and addressed their relative importance in considering the diagnosis of MS. All cases showed perivascular lymphocytic inflammation with variable amounts of macrophage infiltration, necrosis, and edema. The hypercellularity of the lesions and the presence of atypical reactive astrocytes with mitotic figures were the disturbing features that might have led to the erroneous diagnosis of an astrocytic neoplasm. Immunohistochemistry for astrocytic (glial fibrillary acidic protein) and macrophage (HAM-56) markers are helpful in evaluating biopsies. Our results emphasize the need to perform special stains (i.e., for myelin and axons) that demonstrate myelin loss and relative preservation of axons and allow a correct diagnosis.

Hypercalcemic crisis in pregnancy associated with excessive ingestion of calcium carbonate antacid (milk-alkali syndrome): successful treatment with hemodialysis.

Severe hypercalcemia, a potentially life-threatening condition, has been reported rarely during pregnancy. A patient with hypercalcemic crisis associated with excessive ingestion of absorbable calcium antacid was treated successfully with hemodialysis as well as other therapeutic measures, such as saline diuresis. This acute therapy resulted in long-term normalization of maternal calcium levels. The fetus, who exhibited a low biophysical score during the initial admission, was delivered a month later and had an uncomplicated neonatal course. Acute hemodialysis can be an effective, rapid, and safe method of lowering serum calcium levels in pregnant patients with severe hypercalcemia unresponsive to other medical therapies.