Ernest E. Lack

Histopathology of benign versus malignant sympathoadrenal paragangliomas: Clinicopathologic study of 120 cases including unusual histologic features

The clinical and pathologic features of 120 adrenal and extraadrenal paragangliomas were studied in an attempt to identify features which might predict malignant behavior. Clinical follow-up was obtained in 98 cases (82%); 64 tumors were clinically benign, and 34 were malignant as evidenced by regional or distant metastases and/or extensive local invasion. Thirty-two of the 34 malignant tumors (94%) were functionally active. Features noted more frequently in malignant tumors included male predominance (74%; P2 [two-sided P value] = .002), extraadrenal location (52%; P2 less than .0001), greater tumor weight (mean 383 g versus 73 g for nonmalignant tumors), confluent tumor necrosis, and the presence of vascular invasion and/or extensive local invasion. Intracytoplasmic hyaline globules were seen in 59% and 32% of benign and malignant tumors, respectively (P2 = .001). Logistic regression analysis of 16 nonhistologic and histologic parameters showed four of them to be most predictive of malignancy--extraadrenal location, coarse nodularity of the primary tumor, confluent tumor necrosis, and absence of hyaline globules. Most malignant paragangliomas had two or three of these features (71%), while 89% of benign tumors had only one (or none; P less than .0001). According to the statistical model developed, there was better than a 95% probability that more than 70% of tumors could be classified correctly on the basis of the four factors indicated. Although limitations still exist, results of this study provide some basis for evaluating malignant potential of these tumors.

The effect of Zollinger-Ellison syndrome and omeprazole therapy on gastric oxyntic endocrine cells

In 1983, all trials of omeprazole in humans were stopped because rats given the drug developed gastric endocrine cell hyperplasia and carcinoid tumors. Further studies in rats showed that drug-induced achlorhydria and hypergastrinemia caused these changes. Because data in humans are limited, we compared the numbers of endocrine cells, as judged by silver staining (argyrophilia), in the gastric mucosa of patients with Zollinger-Ellison syndrome, who are hypergastrinemic, and in normogastrinemic patients with idiopathic acid-peptic diseases. In addition, we analyzed the number of gastric endocrine cells in patients with Zollinger-Ellison syndrome given omeprazole for up to 3 years. Patients with Zollinger-Ellison syndrome had 15.7% +/- 6.9% argyrophil cells in biopsies of gastric oxyntic mucosa, and patients with idiopathic acid-peptic disease had 7.8% +/- 2.3% (P less than 0.01). In patients with Zollinger-Ellison syndrome, the percentage of argyrophil cells was not related to serum gastrin concentration, duration of symptoms, time since diagnosis, basal or maximal acid output, extent of tumor, or age. There was a tendency for patients with multiple endocrine neoplasia type 1 to have a greater percent of argyrophil cells than patients with sporadic Zollinger-Ellison syndrome. Considering the biopsies from both normogastrinemic and hypergastrinemic patients, there was a significant relationship between the percentage of argyrophil cells and the serum concentration of gastrin (P less than 0.01). Patients with Zollinger-Ellison syndrome given omeprazole for up to 3 years developed no significant changes in percentage of argyrophil cells, no carcinoid tumors, and no changes in serum concentrations of gastrin. The present study shows that patients with Zollinger-Ellison syndrome have an increased percentage of argyrophil cells in oxyntic mucosa and that omeprazole does not increase this percentage. In periods of up to 3 years, omeprazole had no effects on gastric morphology in patients with Zollinger-Ellison syndrome.

Intra-abdominal 'angiosarcomatosis' report of two cases after pelvic irradiation

Angiosarcomas account for only 1% to 2% of all soft tissue sarcomas with the most common site of origin being skin and subcutaneous tissue particularly on the face and scalp of elderly patients. These neoplasms rarely arise in the small or large bowel with presentation as diffuse abdominal “angiosarcomatosis.” Two patients are reported who were treated with adjuvant pelvic irradiation for gynecologic malignancies (squamous cell carcinoma of the cervix and adenocarcinoma of the endometrium) in whom diffuse abdominal angiosarcoma of intestinal origin developed 7 and 18 years later. Both patients were treated with standard megavoltage irradiation. The clinical course was rapid in both cases with death due to intra‐abdominal hemorrhage and bowel obstruction. The pertinent literature relating to the association between radiation therapy and subsequent development of angiosarcomas of soft tissues and other sites is briefly reviewed.

Case report 560

A 75-year-old white woman presented with a 40-year history of a slowly growing bony mass on the anterior surface of the left tibia. Several months prior to admission she suffered minor trauma to the leg and since then, she has had increasing pain and some paresthesia in her foot. Physical examination revealed a fixed, nontender 7 cm x 15 cm bony mass on the anterior surface of the left tibia. The neuro-vascular status of her foot was intact. Roentgenograms and tomogram of the left tibia showed a large, sessile lobulated juxta-articular mass of dense cortical bone, without obvious cortical invasion, soft tissue mass, or periosteal reacton. No cleavage plane was seen between the lesion and the cortex of the tibia (Fig. 1 A and B). A C T scan confirmed these findings (Fig. 2). Angiography showed the lesion to be fairly vascular without the typical features of malignant growth. A bone scan showed increased uptake of tracer in the lesion (Figs. 3 A and B, 4). The patient then underwent a surgical procedure.

Müllerian adenosarcoma of the uterus with pure angiosarcoma: Case report

We report a case of müllerian adenosarcoma of the uterus with benign endometrioid epithelial component and histologically pure angiosarcoma. This seemingly unique sarcomatous ingredient, among uterine and extrauterine adenosarcomas reported in the English literature, is considered to be a homologous component of the tumor. The patient, a 71-year-old woman with a several-month history of intermittent vaginal bleeding, was found at abdominal hysterectomy to have a large polypoid intrauterine tumor with superficial extension into the myometrium. The patient was treated with 6,000 rad of adjuvant radiation but died a few months after of what clinically appeared to be massive abdominal bleeding. The literature regarding uterine tumors classified as angiosarcoma or malignant hemangioendothelioma is briefly reviewed.

Adrenocortical neoplasms in childhood and adolescence: Analysis of prognostic factors including DNA content

Thirty-two adrenocortical neoplasms in children and adolescents were evaluated for prognostic factors including clinical and morphological parameters and DNA ploidy. The patients were segregated into two groups according to clinical outcome: group A, represented by patients with clinically benign neoplasms (n = 15), and group B, patients with clinically malignant tumors as evidenced by local recurrence, metastases, or fatal outcome (n = 17). Clinical and morphological parameters in these two groups were evaluated using appropriate statistical methods. Parameters with a significant predictive value in terms of prognosis were age [p = .04], tumor size (p = .0003), median tumor weight (p = .0001), mitotic count (p = 0.04), and 25% tumor necrosis or more (p = .03). Twenty-three cases were studied for DNA ploidy: 10 cases by image analysis and 13 by both image analysis and flow cytometry. By ploidy analysis, 17 of 23 cases—12 of 14 in group A and 5 of 9 in group B—were found to be aneuploid. Multiple aneuploid peaks were found in 5 of 23 cases—4 of 14 cases in group A and 1 of 9 cases in group B. In tumors studied by both image analysis and flow cytometry, there was no discrepancy between results of ploidy analysis. There was no statistically significant association demonstrated between clinical outcome and DNA ploidy pattern. DNA ploidy heterogeneity, characterized by multiple aneuploid populations of cells, was also detected in both benign and malignant neoplasms. Based on our results, aneuploidy is relatively frequent in pediatrie adrenocortical tumors and does not appear to have predictive value for biological behavior.

Microanatomy of Vagal Body Paraganglia in Infancy Including Victims of Sudden Infant Death Syndrome

The microanatomy of vagal body paraganglia (VBP) in the cephalic segment of both vagus nerves was evaluated in an autopsy study of 32 infants one year of age or younger. The study group included 14 victims of sudden infant death syndrome (SIDS) and 18 non-SIDS cases. VBP in both groups were located at or below the lower border of the ganglion nodosum, and were histologically identical to carotid body chemoreceptors, although spatially dispersed and much smaller in size. Using a combined step and serial sectioning technique, there were no significant differences between the two groups (i.e. SIDS vs. non-SIDS) with regard to microanatomy, number, distribution and size of VBP. The proportion of chief and sustentacular cells was similar to carotid body paraganglia. For the study group as a whole, VBP were present in 89% of vagus nerves, and were typically multiple with good correlation between the number of separate paraganglia on the two sides. Small collections of ectopic parathyroid chief cells were identified in 6% of nerve segments, and histologically should be distinguished from VBP. Although the microanatomy of VBP in the SIDS group was identical to that of non-SIDS, one cannot exclude an underlying functional abnormality with autonomic malregulation.

Recommendations for reporting of tumors of the adrenal cortex and medulla

Abstract The Association of Directors of Anatomic and Surgical Pathology has developed recommendations for the surgical pathology report for common malignant tumors. The recommendations for tumors of the adrenal are reported herein.

Diffuse hemorrhagic gastroenteropathy: Report of a new entity

An apparently novel entity, diffuse hemorrhagic gastroenteropathy (DHG), in a 70-year-old female who had an unremitting course of chronic gastrointestinal blood loss for 3 years requiring transfusion of more than 200 units of packed red blood cells over this period is reported here. Endoscopy showed diffusely hemorrhagic mucosa in the stomach, duodenum, and small bowel. Full-thickness biopsy of the stomach and small intestine revealed luminal narrowing of capillaries and post-capillary venules within the lamina propria due to swelling and some proliferation of the endothelial cells with margination and emigration by neutrophils as well as partial occlusion of some vessels by fibrin thrombi. DHG may represent a new entity characterized by mucosal hemorrhage due to local mucosal ischemia of the gastrointestinal tract secondary to a small vessel vasculopathy apparently restricted to this site.

Open-quiz solution Case report 610

In summary, a case of amyloid arthropathy of the left ankle in a 64-year-old patient with multiple myeloma is presented. The asymmetric presentation and site of deposition in the ankle were taken to be unusual. Involvement of both hips and the right shoulder was also suspected on subsequent evaluation. The patients history and MR studies were essential in establishing the preoperative diagnosis. The extent of involvement, destruction of underlying cartilage, associated effusion and tenosynovitis were optimally defined by the MR images. The MR images also provided preoperative guidance in determining the approach and optimum site of biopsy.