Sören Schröder

Intraductal mucin-hypersecreting neoplasms of the pancreas: A clinicopathologic study of eight patients

Intraductal mucin-hypersecreting neoplasms of the pancreas with extreme dilatation of the main duct were studied in eight patients. They included five men and three women, aged 47-85 years. Five patients had a history of symptoms mimicking pancreatitis; four developed steatorrhea and/or diabetes. At endoscopic retrograde pancreatography, five patients showed an open ampulla filled with mucin, and six patients showed patchy filling defects in the ectatic main duct. Morphological examination showed extreme dilatation of the entire pancreatic duct in six patients and its tail segment in two patients. The duct segments filled with viscous mucin were lined by well-differentiated mucin-secreting cells, forming papillary foldings and occasionally showing cellular atypia. None of the patients had invasive tumor or metastasis. Six patients whose lesions were resected are alive and doing well (mean follow-up, 5.5 years). It is concluded that intraductal mucin-hypersecreting neoplasm is a pancreatic tumor with favorable prognosis. Because it shares many features with intraductal papillary neoplasm, a common pathogenesis of these pancreatic tumors is suggested.

Prognostic factors in medullary thyroid carcinomas. Survival in relation to age, sex, stage, histology, immunocytochemistry, and DNA content

Patients with medullary thyroid carcinomas (MTC) were analyzed according to age, sex, and tumor stage. In addition, the MTC were screened for the predominant histologic pattern, immunocytochemical spectrum (60 tumors), and DNA content (DNA cytophotometry and DNA flow cytometry, 25 tumors). These findings were correlated with follow‐up data available for 45 of these patients. Forty‐eight percent of the tumors revealed a polygonal cell pattern, whereas 22% showed spindle‐cell predominance. All tumors contained cytokeratin, chromogranin A, and calcitonin (CT). Calcitonin gene‐related peptide (CGRP) was present in 92%, carcinoembryonic antigen (CEA) in 77%, neuron‐specific enolase (NSE) in 75%, and vimentin in 53% of cases. Positivity for neurotensin, somatostatin, neurofilaments, bombesin, and alpha human chorionic gonadotropin (a‐hCG) and serotonin ranged between 3% and 27%. All MTC were negative for substance P, adrenocorticotropic hormone (ACTH), thyroglobulin (TG), or S‐100 protein. Local recurrences and regional lymph node metastases revealed identical staining patterns as the primaries. Prognosis of MTC was found not to be related to histologic features (dominant architectural pattern, cellular shape, presence of amyloid deposits) or immunocytochemical pattern. Instead, survival was significantly correlated to age, sex, and stage of disease. The best prognosis was seen in women younger than 40 years and revealing an early stage of disease. DNA measurements added valuable information in assessing the prognosis of MTC.

Surgical pathology of gastrinoma. Site, size, multicentricity, association with multiple endocrine neoplasia type 1, and malignancy

Specimens from the pancreas and duodenum of 26 patients with sporadic Zollinger‐Ellison syndrome (ZES) and 18 patients with multiple endocrine neoplasia type 1 (MEN‐1) and hypergastrinemia (17 with ZES) were screened immunocytochemically for gastrinomas. Location, size, multicentricity, and malignancy of the gastrinomas were evaluated. The MEN‐1 patients had gastrinomas in the duodenum (nine of 18), pancreas (one of 18), and periduodenal lymph nodes (two of 18). No gastrinoma was identified in six patients. Most duodenal gastrinomas were multiple (five of nine) and smaller than 0.6 cm (six of nine). Lymph node metastases were present in eight of 12 patients. All 26 patients with sporadic ZES had a solitary gastrinoma; 14 were found in the pancreas and had a diameter greater than 2 cm. Ten patients had a duodenal gastrinoma, two with a diameter less than 0.6 cm. In two patients, only periduodenal “lymph node gastrinomas” were detected. Eighteen of the sporadic gastrinomas were malignant. These results suggest that duodenal location and multicentricity of gastrinomas are associated with the MEN‐1 syndrome, and solitary gastrinomas, either in the pancreas or the duodenum, are predominantly seen in sporadic ZES.

Diffuse sclerosing variant of papillary thyroid carcinoma. S-100 protein immunocytochemistry and prognosis.

The recently published second edition of the WHO classification of thyroid tumours describes the diffuse sclerosing papillary carcinoma (DSPC) as a specific variant of papillary thyroid cancer (PC). Besides several histological hallmarks, this rare tumour is characterized by its occurrence in young individuals and is thought to have a less favourable prognosis than PC in general. The observations on two examples of this tumour presented herein, however, are at variance at this assumption. The neoplasms occurred in a 10 year old girl and a 34 year old woman. Each time, diffuse involvement of both thyroid lobes and bilateral cervical lymphadenopathy were seen. In one case, the carcinoma extended into the cervical soft tissue. Follow-up disclosed both patients to be without evidence of disease 2 and 13 years, respectively, after thyroid surgery. Immunocytochemically, both thyroid primaries as well as 7 other cases of DSPC reported in the literature showed dense accumulations of S-100 protein positive dendritic/Langerhans cells. Such infiltrations have been demonstrated to be correlated with a benign clinical course of PC. It is thus suggested that DSPC behaves similarly or even less aggressively than PC in general, at least if prominent Langerhans cell infiltration is present.

Sonographic tissue characterisation in thyroid gland diagnosis. A correlation between sonography and histology.

SummaryPunch biopsy was carried out in 32 cases in which thyroid gland changes were sonographically classified as being of homogeneously normal or of homogeneously low echogenicity. Mean follicle lumen size was morphometrically determined from the histological sections. This produced a significant, positive correlation between echogenicity and follicle size. The mean follicle lumen diameter in so-called echonormal structures was 67 µm (SD±23 µm), and (SD±8 µm) in low-echogenic lesions. Thus normal echogenicity represented a normofollicular or macrofollicular structure, while a low echogenicity pattern indicated a microfollicular or solid tissue structure. The structure of thyroid carcinomas is not homogeneously normofollicular or macrofollicular; in exceptional cases they are such only focally. Therefore the significance of these findings lies in the exclusion of malignancy in the event of homogeneously normal echogenicity.

Mucinous Nonneoplastic Cyst of the Pancreas: A Novel Nonneoplastic Cystic Change?

Cystic lesions and neoplasms of the pancreas are uncommon, but they are of special interest because they can usually be cured by resection. During the last decade, the spectrum of these tumors has increased considerably. We present a series of five cystic lesions of the pancreas that differ from all categories described so far. The patients affected by these tumors were three men and two women (mean age, 57 y). Four lesions were unifocal and involved the head of the pancreas; one was multifocal and involved the pancreatic head and tail. Grossly, these tumors presented as unilocular or multilocular thin-walled cysts that contained turbid fluid, or, in two cases, blood, and lacked any communication with the duct system. Microscopically, the cysts were lined by cuboidal to columnar mucin-producing cells, supported by a small band of dense fibrous stroma. Immunocytochemically, the epithelial cells were positive for cytokeratins 7, 8, 18, 19, and 20 (except one), and Ca 19-9 but were negative for trypsin, CEA, synaptophysin, chromogranin A, calretinin, and α-inhibin. In four of the five lesions, the epithelial cells expressed MUC5AC, and in one of the five, MUC1. MUC2 and MUC6 were not expressed in any of the lesions. The stromal cells lacked the nuclear progesterone positivity that is typical of mucinous cystic neoplasms. During a mean follow-up period of 2 years, there were no recurrences or cases of malignant transformation after resection. The results suggest that these cystic lesions are distinct from mucinous cystic neoplasms, the most important entity in the differential diagnosis. Because they may represent a nonneoplastic cystic change of the pancreas, we propose the descriptive term mucinous nonneoplastic cyst for these tumors of unknown pathogenesis.

Carcinoembryonic antigen and nonspecific cross-reacting antigen in thyroid cancer. An immunocytochemical study using polyclonal and monoclonal antibodies.

AbstractThe expression of carcinoembryonic antigen (CEA) and related antigens in formalin-fixed paraffin-embedded tissues of 200 primary thyroid carcinomas and 22 nonmalignant thyroid lesions was studied immunocytochemically by using three monoclonal antibodies recognizing different epitopes on CEA and related antigens. The monoclonal antibody 431/31 defined epitope (present only on CEA) was found exclusively on 77% of the medullary thyroid carcinomas and on 100% of hyperplastic C-cell nodules adjacent to infiltrating C-cell carcinomas. The monoclonal antibodies 374/14 and 250/183, defining epitopes present on CEA and the nonspecific crossreacting antigens 95 or 55, reacted with 88% and 82%, respectively, of the medullary carcinomas, but labelled also many nonmedullary thyroid carcinomas (follicular 34% and 18%, papillary 42% and 30%, anaplastic 18% and 13%), follicular adenomas, and nonneoplastic follicle- cell lesions. Similar results were obtained by using a polyclonal CEA antiserum preabsorbed with lyophilized spleen tissue. The findings suggest that nonspecific cross-reacting antigens can be expressed by all types of thyroid carcinoma, while CEA is present only in C-cell tumors of this organ.

The encapsulated papillary carcinoma of the thyroid: a morphologic subtype of the papillary thyroid carcinoma

Of a total of 182 papillary neoplasms, 25 lesions (average size, 3.1 cm) were classified on cytologic and histologic grounds as being encapsulated carcinomas. In eight of these tumors (32.2%) cervical lymph node metastasis and/or intraglandular lymphatic spread was observed. It was not possible to separate on histologic grounds those that did metastasize from those that did not. The excellent prognosis for the encapsulated variant of papillary thyroid carcinoma was confirmed by a long follow‐up period in which no evidence of recurrences or further metastasis was registered as compared with the time of initial diagnosis, whatever the mode of therapy. On the basis of these findings, the encapsulated papillary carcinoma can be regarded as an early or slowly growing form of the papillary thyroid carcinoma.

Immunocytochemistry in adrenocortical tumours: a clinicomorphological study of 72 neoplasms.

Surgical specimens of 72 adrenocortical tumours (ACTs) were investigated. Histologically, 57 tumours were classified as adenomas and 15 as carcinomas. In 9 of the latter cases, distant metastases and/or lethal outcome of disease was recorded. Immunocytochemistry showed only 2 ACTs to be positive for cytokeratin and 6 for vimentin. None of the 72 tumours showed argyrophilia or immunoreactivity for epithelial membrane antigen (EMA), S-100 protein, chromogranin A, Leu 7 or Leu-M1, while 31 cases exhibited positivity on inimunostaining with a polyclonal antiserum against synaptophysin. All 72 ACTs were immunoreactive with the recently described antibody D11. Thus the panel of antibodies described here could not discriminate between adenomas and carcinomas or between carcinomas with aggressive and indolent behaviour. Immunostaining with D11 and for EMA and Leu-M1 may help to distinguish ACTs from phenotypically similar lesions of different histogenesis.

Adenolipoma (thyrolipoma) of the thyroid gland report of two cases and review of literature

Rare fat cell-containing adenomas (adenolipomas) found in the thyroid gland of two patients are presented. Previously documented cases are reviewed. An origin from embryonic rests for these tumours as for diffuse lipomatosis of the thyroid is discussed.