Pancreatic tumors in multiple endocrine neoplasia type 1: Clinical presentation and surgical treatment

Among 33 patients with endocrine pancreatic tumors due to multiple endocrine neoplasia type 1 (MEN-1), 19 (58%) patients had hypergastrinemia, 7 (21%) patients had hyperinsulinism, and 7 (21%) patients had clinically non-functioning lesions. At least one gross tumor was found in all patients undergoing pancreatic surgery, including those with negative localization studies prior to operation. The patients also had additional macroscopic tumors as well as numerous microadenomas, and the lesions frequently were positive for immunostaining with multiple hormones, mainly pancreatic polypeptide, insulin, glucagon, and somatostatin. Duodenal endocrine lesions were found in 4 of 5 investigated patients and stained with gastrin and somatostatin antibodies. Distal, mainly subtotal pancreatic resection, was performed in 18 patients, eventually combined with caput tumor enucleation or duodenotomy, while a few patients underwent only tumor enucleation or a Whipple procedure. The long-erm outcome of operation was most favorable in patients with hyperinsulinism; only 1 patient had clinical recurrence. Patients with hypergastrinemia experienced only transitory lowering of serum gastrin values after pancreatic surgery and 47% of them had or developed metastases. Such tumor spread was seen in 57% of the patients with non-functioning lesions. Nine patients died from progressive tumor disease during follow-up. Consistent with previous studies, we found that surgery is indicated in MEN-1 patients with hyperinsulinism even if a lesion is not visualized by radiology. In addition, these indications should be extended to also include patients with only biochemical markers of disease, including elevations of gastrin, as these indicate the presence of gross tumors. This strategy should be applied especially in patients with aggressive family histories to possibly reduce the risk of malignant tumor progression.

---

Resumen Entre 33 individuos con tumores pancréaticos endocrinos como componente del síndrome de neoplasia endocrina múltiple tipo 1 (NEM-1), 19 pacientes (58%) tenían hipergastrinemia, 7 (21%) hiperinsulinismo y 7 (21%) lesiones clínicas no funcionantes. En la totalidad de los pacientes sometidos a cirugía pancreática fue hallado por lo menos un tumor, incluso en aquellos con examenes de localización negativos anteriores a la operación. Estos pacientes también albergaban tumores macroscópicos, así como numerosos microadenomas; con frecuencia las lesiones demostraron inmunocoloración con diferentes hormonas, principalmente polipéptido, insulina, glucagón y somatostatina. Se encontraron lesiones endocrinas duodenales en 4 de cada 5 pacientes investigados, las cuales colorearon con gastrina y anticuerpos a la somatostatina. Se practicó resección pancreática distal (principalmente resección subtotal) en 18 pacientes, eventualmente combinada con enucleación del tumor (cuando éste se hallaba ubicado en la cabeza del páncreas) o duodenectomía; solamente unos pocos pacientes fueron sometidos a simple enucleación del tumor o al procedimiento de Whipple. El resultado a largo plazo fue más favorable en los pacientes con hiperinsulinismo, puesto que sólo uno presentó recurrencia clínica. Los pacientes con hipergastrinemia exhibieron apenas una disminución transitoria de los valores de gastrina sérica luego de la cirugía pancreática. Cuarenta y siete por ciento del conjunto tuvo o desarrolló metástasis, en tanto que la extensión local del tumor se presentó en 57% de los casos con lesiones no funcionantes. Nueve pacientes murieron por progresión de la neoplasia en el curso del seguimiento. En acuerdo con sugerencias previas, se considero quo la cirugía está indicada en pacientes con NEM-1 e hiperinsulinismo, aún en los casos en que no se visualiza radiológicamente la lesión, pero que la indicación puede ser ampliada para incluir también pacientes con sólo marcadores bioquímicos, tales como niveles elevados de gastrina, indicativos de la presencia de tumores macroscópicos. Esta estrategia debe ser aplicada principalmente en aquellos pacientes con historia familiar agresiva, con lo cual tal vez se reduce el riesgo de progesión maligna del tumor.

---

Résumé Parmi 33 patients ayant une tumeur pancréatique endocrine due à une néoplasie endocrine multiple de type 1 (MEN-1), 19 (58%) avaient une hypergastrinémie, 7 (21%) un hyperinsulinisme et 7 (21%) une lésion cliniquement muette. On a mis en évidence au minimum une grosse tumeur chez tous les patients, y compris chez ceux dont les examens préopératoires de dépistage tumoral étaient négatifs. Les patients étaient également porteurs de tumeurs macroscopiques et de nombreux microadénomes. Les lésions montraient souvent un immunomarquage positif pour de multiples hormones, principalement le polypeptide pancréatique, l'insuline, le glucagon et la somatostatine. Des lésions endocrines duodénales furent retrouvées chez 4 des 5 patients explorés; elles montraient un immunomarquage avec les anticorps angigastrine et anti-somatostatine. Une résection pancréatique distale, le plus souvent subtotale, a été réalisée chez 18 patients. Elle était éventuellement complétée par une énucléation tmorale de la tête ou par une duodénotomie. Peu de patients ont bénéficié d'une simple énucléation ou d'une intervention de Whipple. L'évolution postopératoire à long terme a été plus favorable en cas d'insulinome puisque seul un patient a eu une récidive clinique. Les patients atteints de gastrinome n'ont présenté que transitoirement une diminution des taux sériques de gastrine après la chirurgie pancréatique. Quarante sept pour cent de ces patients avaient ou ont développé des métastases contre 57% des patients porteurs de lésions sans traduction clinique. Neuf patients sont décédés en raison de l'extension tumorale au cours du suivi. Conformément à des suggestions antérieures, la chirurgie semble indiquée chez les patients atteints de MEN-1 avec hyperinsulinisme même si la radiologie ne visualise pas de lésion. Mais cette indication peut être élargie aux patients dont seuls les paramètres biologiques sont en faveur d'une grosse tumeur (dont l'hypergastrinémie). Cette stratégie pourrait convenir particulièrement aux patients ayant des antécédents familiaux importants; elle permettrait peut-être de réduire le risque d'extension tumorale.

---

---

Presented at the International Association of Endocrine Surgeons in Stockholm, Sweden, August, 1991.     

Method for Dissection of Mesenteric Metastases in Mid-gut Carcinoid Tumors

With adequate medical management the midgut carcinoid tumor generally is an indolent malignancy associated with substantial life expectancy and appreciable life quality, even in the presence of liver metastases and significant tumor burden. Abdominal complications may occur in this entity of carcinoids owing to entrapment of intestines and encasement of mesenteric vessels by mesenteric metastases and associated marked mesenteric fibrosis. This may be the cause of abdominal pain, disabling diarrhea, weight loss to the extent of malnutrition, and eventually the risk of death with acute or chronic intestinal obstruction or intestinal gangrene. Operative removal of the mesentericointestinal lesion is often indicated to prevent or treat these complications but may be technically difficult when mesenteric metastases extend in the vicinity of major vessels in the mesenteric root. At laparotomy 56 patients with advanced midgut carcinoids underwent removal of the mesenteric tumor with a method for preserving the mesenteric vessels. This was feasible by mobilizing and releasing the right colon and mesenteric root from posterior adhesions, identifying the mesenteric artery below the pancreas, and free-dissecting this artery on the tumor capsule in the mobilized mesentery. Dissection was successful even with tumors initially judged inoperable unless tumor growth completely surrounded the mesenteric vessels or extended retroperitoneally. One patient was subjected to distal intestinal artery bypass. Symptom relief was been substantial and often of long duration after mesenteric tumor removal in patients who prior to surgery often had threatening intestinal ischemia. Patients with advanced midgut carcinoids may benefit markedly from dissectional removal of mesenteric tumors, which (conceivably better than conventional wedge resection) preserves the length of the remaining intestine.     

Measurement of salivary cortisol – effects of replacing polyester with cotton and switching antibody

Stable performance between‐runs are essential in longitudinal studies and when different studies are being compared. However, changes in analytical kits and laboratory material occur and have the potential to threaten analytical stability. In the present case, we examined how salivary cortisol measurements in our laboratory were affected by: 1) changes in the tampon material and 2) changes in the antibody of the analytical kit. In study 1, saliva from healthy subjects (n = 19) was split and spiked to Salivette^® polyester and cotton tampons, respectively, and treated as ordinary samples before being analysed for cortisol using a Spectria RIA kit for cortisol. In study 2, 68 anonymous saliva samples were analysed with the Spectria Cortisol RIA kit both before and after the manufacturer changed the antibody. The change from polyester to cotton tampons reduced the measured concentration of salivary cortisol by 62?%. A difference of 12?% between the two runs with different antibodies could not be attributed to differences in storage or in thawing and freezing of samples. To conclude, both a change in the material of the Salivette used for collecting saliva samples as well as a change of antibody in a kit can have a major impact on measurements, as illustrated here for concentrations of cortisol in saliva. It is therefore recommended always to check that the analysis stays in statistical control in one's own laboratory when changes are made, even if the manufacturer reports that the changes should have no effects.     

Family Dynamics and Child Abuse and Neglect in Three Finnish Communities

The purpose of this study was to describe family dynamics of Finnish families in which there is abuse or neglect of a child in that family. One adult from 41 families with recognized child abuse/neglect completed the Family Dynamics Measure II (FDM II) consisting of six dimensions of family life, and answered questions about problems, changes and illnesses. Nurses and social workers in community health care and social agencies identified potential subjects in their agencies and collected data. Four of the six dimensions of the FDM II reached statistical significance ( p =. 05) with child age and number of children, education, marital status, and age of respondent. They are: Flexibility vs. rigidity, Stability vs. disorganization, Clear vs. distorted communication, and Role reciprocity vs. role conflict. FDM II is useful in identifying several of the less positive family dynamics dimensions in abuse:neglect families. Conclusions for clinical management cannot be drawn at this time.     

Behavioral goal assessment in patients with persistent musculoskeletal pain

Identification and achievement of behavioral goals is an important issue in pain rehabilitation. For this dual purpose, we developed a patient-specific clinical tool—the Patient Goal Priority Questionnaire (PGPQ). Using the PGPQ, this study identifies patients' behavioral goals for physical therapy (PT) in a primary health care sample of patients with persistent musculoskeletal pain. In addition, this study examines the concurrent validity of the PGPQ in relation to a generic measure of disability, the Pain Disability Index (PDI). In all, 197 subjects participated in the study. The behavioral goals differed among the patients, comprehending several everyday activities and behaviors, and thus an individualized and behavioral focus was relevant for these patients. The PGPQ was negatively and moderately correlated with the PDI, indicating patient-specific properties of the new instrument. An elaborated version of the PGPQ can serve (a) as a clinical tool for identification of the patient's priorities of behavioral goals for PT, (b) as a clinical tool for collaborative formative evaluation during treatment, and (c) as a complementary measure in research for assessment of clinically significant changes related to behavioral performance.     

A comparison between flexible electrogoniometers,inclinometers and three-dimensional video analysis system for recording neck movement

This study compared neck range of movement recording using three different methods goniometers (EGM), inclinometers (INC) and a three-dimensional video analysis system (IMG) in simultaneous and synchronized data collection. Twelve females performed neck flexion-extension, lateral flexion, rotation and circumduction. The differences between EGM, INC, and IMG were calculated sample by sample. For flexion-extension movement, IMG underestimated the amplitude by 13%; moreover, EGM showed a crosstalk of about 20% for lateral flexion and rotation axes. In lateral flexion movement, all systems showed similar amplitude and the inter-system differences were moderate (4–7%). For rotation movement, EGM showed a high crosstalk (13%) for flexion-extension axis. During the circumduction movement, IMG underestimated the amplitude of flexion-extension movements by about 11%, and the inter-system differences were high (about 17%) except for INC–IMG regarding lateral flexion (7%) and EGM–INC regarding flexion-extension (10%). For application in workplace, INC presents good results compared to IMG and EGM though INC cannot record rotation. EGM should be improved in order to reduce its crosstalk errors and allow recording of the full neck range of movement. Due to non-optimal positioning of the cameras for recording flexion–extension, IMG underestimated the amplitude of these movements.