Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a rare recently described entity. Only three examples have been documented, one of which showed evidence of malignant transformation. We report an additional four cases and update the literature. With the exception of an 82-year-old man, all patients have been under 30 years of age. The temporal lobe was involved in three cases and cerebellum in another three. Radiologic features were those common to PXA and GG. Histologically, all were "collision tumors" composed of abutting, although spatially distinct, PXA and GG components. In two cases, the second element was only recognized at reexcision or recurrence. Histologic anaplasia, always in the PXA component, was evident as brisk mitotic activity and/or necrosis in five cases. Of the seven patients, one died of disease 17 years after the onset of seizures and after multiple recurrences, the last of which largely resembled glioblastoma. We conclude that the composite PXA-GG is a rare neoplasm that shares many features of its individual components. In addition to its temporal lobe predilection, the cerebellum is frequently affected. As when it occurs in isolation, the PXA component of composite PXA-GG possesses the potential for malignant transformation.
Fifteen oncocytic (oxyphilic granular cell) neoplasms of the salivary glands collected from three large university medical centers are presented. The histologic and ultrastructural criteria that characterize oncocytes are presented and electron micrographs of two of our cases are discussed. Included in the series are 10 benign oncocytomas, two malignant oncocytomas and three benign, oncocytic pleomorphic adenomas. The clinical and pathologic features of benign oncocytomas are reviewed, with special attention to the variety of gross and microscopic changes present. The literature on malignant oncocytomas is critically reviewed, diagnostic criteria are evaluated, and a unique case is discussed in detail. Reference is made to a group of neoplasms that we designate as benign locally aggressive oncocytomas. The problem of differential diagnosis between oncocytoma and bening, oncocytic pleomorphic adenoma is considered.
Twenty-four cases of signet-ring cell carcinoma of the breast are presented. These represented 4.5% of 535 cases of surgically treated breast cancer at Indiana University Medical Center. Twelve cases were associated with ductal carcinoma, nine with lobular carcinoma, one with colloid carcinoma, and four were pure signet-ring cell carcinoma. The mortality rate, incidence of axillary lymph nodal metastases, and number of involved lymph nodes were greater in cases of signet-ring cell carcinoma than with other forms of mammary carcinomas without signet-ring cells. It is proposed that signet-ring cell carcinoma can manifest as a pure lesion or as a variant of ductal, lobular, or colloid carcinoma, and that it is an aggressive histologic variant of mammary carcinoma.
Granular cell tumor of the stomach is rare and may occur in association with similar tumors in the skin. Two such tumors were found in association with cutaneous granular cell tumors in two black females. Infiltrating carcinoma of the stomach was suspected at surgery in one case. All of the lesions were typical granular cell tumors on light microscopic examination. Ultrastructural examination showed clusters of tumor cells that were surrounded by basal laminae and axonal structures were present among the tumor cells. S‐100 protein immunoreactivity was also demonstrated in the tumor cells. These findings suggest a Schwannian origin for visceral granular cell tumors.
Light and electron microscopic examination of a goblet cell carcinoid revealed cells with pleomorphic neurosecretory-type granules, cells containing mucin some of which also contained these granules and less differentiated cells lacking the aforementioned features. Recent embryologic and anatomic studies of developing avian and mammalian gut, respectively, show that intestinal APUD cells are probably of endodermal origin. Therefore, mixed carcinoid tumors such as the goblet cell variant could arise in crypt base stem cells. Cancer 44:1700-1706, 1979. HE EXISTENCE OF TUMORS that possess the T morphologic characteristics of two or more discrete embryonic cell systems raises questions concerning the histogenesis and differentiation of these tumors. The goblet cell carcinoid30 is an example of a neoplasm with a mixed phenotype and as such has aroused much interest. Herein we describe the light and electron microscopic morphology of a goblet cell carcinoid. CASE REPORT A 62-year-old diabetic male was admitted with chills, a temperature of 104 F and colicky abdominal pain of 60 hours' duration. Moderate abdominal distention and left lower quadrant tenderness without rebound were noted. Chest and abdominal x-ray revealed linear atelectasis in the left mid-lung field. He was given antibiotics with physical and supportive therapy. Barium enema and colonoscopy were normal. After discharge an intermittent fever (101 F) developed and he was readmitted following collapse and brief loss of consciousness. The blood pressure was 90/60 and the temperature 99.8 F. Slight tenderness was elicited in the left lower quadrant. The hemoglobin was 11.4 g/dl and a neutrophil leucocytosis (WCC 18,000/mm3) was present; fasting blood sugar was 270 mg/dl. The stool contained melena and 2 liters of coffee-ground material were obtained on nasogastric suction. Arterial bleeding without evident ulceration was observed in the second part of the duodenum by endoscopy. The hemo-globin was 7.4 g/dl. At laparotomy, after blood transfusion, a shallow linear ulcer with an active bleeding point was oversewn. About 100 ml of pus was aspirated from an appendiceal abscess which lay between the sigmoid colon and a loop of jejunum and beneath extensive adhesions. The abscess with a partly necrotic appendix and fecalith were removed. A portion of the cecum with appendix stump was excised three months later. Recovery was uneventful and the patient is free of recurrence three years later. MATERIALS AND METHODS Sections of formalin-fixed paraffin-embedded blocks of tissue were stained with hematoxylin-eosin, periodic acid-Schiff, mu-cicarmine, Alcian blue, trichrome, basic fuchsin, naphthol-ASD-chloroacetate ester-ase,I7 Grimelius and Masson-Fontana methods. Immunoperoxidase staining for mur-amidase was performed using Pangalis' method.23 Normal appendix, ileum and an ileal carcinoid were used as controls. Tissue blocks for electron microscopy were taken from the formalin-fixed specimen within 36 hours, postfixed in 3% glutaraldehyde and subsequently processed i...
Two cases of an oncocytic adrenal cortical tumor that contained peculiar cytoplasmic crystalline inclusions in the tumor cells are presented. The patients were 49- and 72-year-old females without clinical and biochemical evidence of adrenal cortical or medullary dysfunction. The adrenal tumors weighed 80 and 200 g each. These crystalline inclusions were present in groups of longitudinal profiles or clusters of crossly cut aggregates. They appeared in clusters of membrane-bound columns. On longitudinal sections, they appeared as rigid rods of homogenous density measuring 36 nm in width, but when they were cut transversely their paracrystalline nature became apparent. They were composed of closely packed microtubules in rectangular blocks. The microtubules measured 12.5 nm with a hollow center measuring 4.2 nm. The inclusions were within the membrane-bound cisterna of rough-surfaced endoplasmic reticulum. The significance of these inclusions is not clearly understood; however, they have been seen only in adrenal cortical tumors and their presence may be helpful in the differential diagnosis of adrenal oncocytic tumors. One patient presented with a tumor in which gross and microscopic appearance was compatible with a pheochromocytoma. This case exhibited an oncocytic appearance and pronounced cellular pleomorphism. Ultrastructural studies were necessary to recognize the tumor cells as cortical cells. The tumor cells contained abundant mitochondria with tubular cristae, paranuclear parallel stacks of granular endoplasmic reticulum, and relatively prominent smooth endoplasmic reticulum. These features are typical of adrenocortical cells. In addition, frequent tumor cells contained the peculiar cytoplasmic inclusions herein described.
A large hepatic neoplasm excised from a 62-year-old man resembled a carcinoid tumor. No endocrine syndrome was present and an alternative primary source of tumor was not found, despite an intensive search. The tumor cells were classified according to the type of electron-dense granules present: ACTH, gastrin (G), bombesin (P), and pancreatic polypeptide (PP) type granules were identified. Immunofluores-cent staining with antibodies to several polypeptide hormones detected PP-containing tumor cells only. This rare tumor is therefore a PP-apudoma; possibly of intrahepatic bile duct origin.
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