Biomedical subjects
Publications and source records attributed to E E Lack.
A 63-yr-old female presented with uncontrollable uterine bleeding. Pathological evaluation revealed a uterine mesenchymal neoplasm which histologically represented a primary osteosarcoma. Immunohistochemistry was utilized to help support the diagnosis. The English literature regarding this rare uterine neoplasm is briefly reviewed.
Two cases of heterotopic tissue involving the gallbladder are presented (one gastric mucosa, the other liver), and the relevant literature is reviewed highlighting problems in differential diagnosis and complications, particularly with regard to gastric mucosal heterotopia. A firm diagnosis of gastric heterotopia is based on the presence of fundic mucosa replete with parietal and chief cells; a clear distinction from intestinal metaplasia should be made, but at times may be difficult. Potential complications include mucosal ulceration, obstruction, and hemorrhage. Of 39 patients presented, about half were 30 years of age or younger, and only nine have had calculi noted in the cholecystectomy specimen. Heightened awareness of gastric heterotopia with separation of smooth-muscle bundles may help to avoid a malignant diagnosis. Hepatic tissue involving the gallbladder that is completely separate from the main part of the liver is an even rarer form of heterotopia that should be distinguished from an accessory lobe.
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Pituitary adenomas rarely are metastatic. Extracranial visceral metastases of prolactinomas were not previously reported. The authors report a case of a 34-year-old man with a prolactin-producing pituitary carcinoma and histologically proven lung metastases. Pathologic examination of the pulmonary spread included electron microscopy and immunohistochemistry; these confirmed prolactin production by the tumor. The patient's presentation at initial diagnosis, disease recurrence, clinical course, management, and response to therapy (with its theoretic basis) are detailed. Despite the use of dopamine analogues (to tolerance and in combination), there was documented intracranial and extracranial disease progression. Possible future therapeutic maneuvers are discussed.
Combined modality treatment has resulted in cure rates of approximately 80% for children with Wilms' tumor. According to the National Wilms' Tumor Studies (NWTS), a group of patients with histologic features of anaplasia or sarcomatous Wilms' tumors (malignant rhabdoid tumors and clear cell sarcomas) were less responsive to vincristine and actinomycin. The survival rate of patients in this group with unfavorable histologic conditions was 54% compared with 90% for those with favorable histologic conditions. We have reviewed 80 consecutive cases of Wilms' tumor treated with a minimum follow-up interval of 5 years. Two pathologists independently reviewed all histologic specimens that were initially classified as having unfavorable histologic conditions and specimens from children with favorable histologic conditions who subsequently relapsed. One of 13 children with favorable histologic conditions had recurrent disease that was found to have unfavorable histologic conditions on rereview. All five patients with sarcomatous Wilms' tumor had a rapidly progressive course. Treatment of eight children with anaplastic Wilms' tumor with vincristine, actinomycin, cyclophosphamide, and abdominal radiation resulted in good disease-free and overall survival rates (5-year survival rate, 87.5%) that were not significantly different from children with tumors having favorable histologic conditions (5-year survival rate, 94%). All children with sarcomatous histologic conditions, however, did not to respond.
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.
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.
Two anaplastic thyroid carcinomas with osteoclast-like giant cells (OCL-GC) are reported. Light microscopically, one case resembled an aneurysmal bone cyst with cellular connective-tissue septae separating cavernous, blood-filled sinuses. The second case had sheets of anaplastic cells and a separate focus of papillary carcinoma without areas of transition. Multinucleate OCL-GC, pleomorphic mononuclear cells, and histiocytoid mononuclear cells with nuclei similar to those within the OCL-GC were seen in each case. With formalin-fixed, paraffin-embedded tissue, the majority of OCL-GC and histiocytoid mononuclear cells in both cases showed immunoreactivity using monoclonal antibodies to vimentin and KP-1, with the latter preparation directed against cells of monocytic/histiocytic lineage. Staining for keratin, epithelial membrane antigen, neuron-specific enolase, chromogranin, calcitonin, and thyroglobulin was negative in all cell types. Ultrastructural examination of one case showed two distinct types of mononuclear cells--one with morphologic characteristics similar to those of the OCL-GC, and another with pleomorphic nuclei and short cytoplasmic extensions joined by poorly formed desmosomes. The findings indicate that the anaplastic thyroid carcinomas studied represent poorly differentiated epithelial tumors infiltrated by reactive OCL-GC of monocytic/histiocytic lineage apparently derived from histiocytoid mononuclear cells via cellular fusion.
A primary leiomyosarcoma of the right adrenal gland is reported in a 49-year-old male who presented with progressive flank pain. This is the second case in the English language literature and the first to have documentation of malignant behavior. The tumor measured 11 cm in diameter and showed marked necrosis with prominent mitotic activity (average 15 per 10 high-power fields). Smooth muscle differentiation was apparent ultrastructurally and confirmed by positive immunostaining for muscle-specific and alpha-smooth muscle actin. Bony metastases developed; following palliative treatment with radiation and chemotherapy, the patient is alive with tumor 9 months later. Origin from smooth muscle associated with the central adrenal vein or its tributaries is proposed.
Flow cytometry and nuclear morphometry were compared with traditional pathologic grading techniques for predicting the course of malignant fibrous histiocytoma of the extremities. Clinical, pathologic, and flow/morphometric variables from 53 cases were tested by Cox regression for prediction of distant recurrence and mortality. Tumor grading based on extent of tumor necrosis was a significant predictor for both disease-free survival (p = .014) and overall survival (p = .003). The fraction of nuclei in the S + G2M segment of DNA histograms was significant for disease-free survival (p = .007), and remained significant (p = .033) in a joint Cox model with necrosis-based grade (p = .004 for the bivariate model). Relative risk for recurrence varied nearly 10x between the 10th and 90th percentiles of grade and (S + G2M)1/2. Overall survival was predicted by a nuclear shape feature termed "R" (p = .000008), the casewise difference (residual) between expected and observed nuclear perimeter as a function of average Feret diameter. In a bivariate Cox model, relative risk of mortality varied 35x between the 10th and 90th percentiles of grade and R. Cytometric and morphometric data contain information about recurrence-free and overall survival beyond that available from more usual clinical and pathologic features. It seems likely that nuclear morphometry, in particular, will prove to be a useful aid for estimating the prognosis of patients with malignant fibrous histiocytoma of the extremities.
Undifferentiated (embryonal) sarcoma of the liver is a primitive mesenchymal neoplasm with predilection for individuals in the first 2 decades of life. In this study (10 boys, 6 girls), children in the age range of 6-10 years were most commonly affected (63%). Clinical features most frequently noted on presentation were abdominal pain or a palpable mass. In two cases there was cardiac involvement caused by invasion of the inferior vena cava with extension into the right atrium and ventricle; both children died of progressive dyspnea from tumor embolization to the lungs. One patient was a member of a kindred with the cancer family syndrome (Li-Fraumeni syndrome). There were 13 tumor-related deaths (86% mortality); on child was alive with recurrent tumor in the upper abdomen. Complete surgical resection was attempted in 10 of 15 children who underwent exploratory laparotomy; 2 were alive and well 1 and 5 years later, whereas 1 patient had a recurrence in the upper abdomen 3 years after diagnosis. Ultrastructural study (five cases) and immunohistochemistry (11 cases) supported a mesenchymal origin for the tumor, but failed to identify any diagnostic immunophenotype or specific line of differentiation. Coexpression of vimentin and cytokeratin was seen in three cases. Prompt detection of this aggressive tumor with complete surgical resection is the key to a successful outcome, but this is very difficult to achieve. Recent experience suggests that aggressive adjuvant chemotherapy may improve survival in some cases.
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Ten patients with soft tissue hemangiomas outside the central nervous system were studied with MR imaging. Eight patients were studied at 1.5 Tesla (T) with T1-weighted and triple echo T2-weighted sequences. Two additional patients were imaged on a 0.5-T system. The MR images were correlated with images from other modalities. Histologic diagnosis was obtained in all cases. It was found that prolonged T2-weighted imaging together with standard spin echo T1 and T2 pulse sequences is a good substitute for contrast-enhanced CT and arteriographic evaluation of soft tissue hemangiomas.
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 patient's 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.
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.
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.
To further characterize the clinicopathologic spectrum of colorectal neuroendocrine neoplasia, 24 carcinomas with neuroendocrine differentiation were subtyped as either small cell neuroendocrine, oat cell variant (six cases), small cell neuroendocrine, intermediate variant (16 cases), or moderately differentiated neuroendocrine carcinoma (two cases). Five oat cell variants, 14 intermediate variants, and two moderately differentiated tumors were studied with antibodies to cytokeratin, vimentin, epithelial membrane antigen (EMA), neuron-specific enolase (NSE), chromogranin (CRG), synaptophysin (SYN), neurofilament, S-100 protein, carcinoembryonic antigen (CEA), and Leu-7. All tumors were immunoreactive for cytokeratin and the majority were also positive for EMA and NSE. Positivity for specific neuroendocrine markers was uncommon, with SYN reactivity noted in one oat cell variant and four intermediate variants, and CRG positivity observed in four intermediate variants and one moderately differentiated tumor. Ultrastructural analysis of four oat cell, eight intermediate, and one moderately differentiated tumor revealed neurosecretory-type, dense-core granules in all lesions, except two oat cell variants studied from paraffin-retrieved material. Hepatic and regional lymph node metastases were noted in five of six oat cell, eight of 16 intermediate, and two of two moderately differentiated tumors. Of 17 patients with follow-up (four oat cell, 11 intermediate, and two moderately differentiated tumors), only two individuals were alive after 1 year. There were no detectable differences in survival or response to treatment between morphologic subtypes. The prognosis of colorectal neuroendocrine carcinoma appears worse than for adenocarcinoma of comparable stage. Their distinction is thus warranted, especially in regard to the intermediate variant and moderately differentiated tumors, which may be potentially misinterpreted as forms of adenocarcinoma.
To assess the value of ultrasound (US), fluoroscopy, and spot radiography in the detection, counting, and measurement of gallstone fragments during lithotripsy, in vitro visibility studies were conducted on fragments from 20 stones. Fluoroscopic visibility was evaluated during and after lithotripsy on 185 fragments placed in an anthropomorphic phantom. Three US experiments were performed on the fragments to study the visibility of fragments as a function of size, the accuracy of the count with large numbers of fragments, and the ability of observers to detect and count fragments larger than both 4 mm and 5 mm. With fluoroscopy, fragment detection rates ranged from 20% (fragments larger than 2.5 mm) to 80% (fragments larger than 4.5 mm). With US, all fragments larger than 1.5 mm were detected, and US was significantly better than fluoroscopy and spot radiography for detection of fragments 2.5 mm or smaller. US was also more accurate than fluoroscopy (11% vs 59% error) in the assessment of the number of fragments. When fragments larger than 4 mm or 5 mm were being counted with US, 92% of the fragments were visualized. The results suggest that US is more accurate for monitoring gallstone lithotripsy than fluoroscopy or spot radiography.