DNA cytophotometry and prognosis in typical and atypical bronchopulmonary carcinoids. A clinicomorphologic study of 100 neuroendocrine lung tumors.

Surgical material obtained from 100 patients with typical carcinoids (TC) and atypical carcinoids (AC) of the lung (including 100 primary, four residual tumors, and four lymph node or distant metastases) was investigated by conventional histology and scanning DNA cytophotometry. Of the 60 TC (96%), 58 exhibited euploid DNA histograms compared with only 20 (50%) of the 40 AC. The morphologic findings were related to the patients' survival (median observation period, 9 years). Statistical analyses disclosed the histologic type of disease (TC versus AC) and the DNA content of tumors (euploid versus aneuploid) to affect prognosis significantly (p < 0.001). Deaths resulting from tumor were exclusively observed among patients with atypical (eight of 40) or DNA aneuploid carcinoids (eight of 22). Six patients were alive with persistent tumor manifestations 3 to 20 years after initial diagnosis, four with DNA diploid primary carcinoids. The presence of lymph node metastases alone was not associated with poor prognosis as long as the primary tumor or the related metastases showed a diploid DNA content. DNA cytophotometry thus might be regarded as an adjunctive prognostic criterion in individual carcinoid cases.

New autosomal recessive lethal disorder with polycystic kidneys type Potter I, characteristic face, microcephaly, brachymelia, and congenital heart defects.

We report on 3 pairs of sibs from unrelated families, who present with polycystic kidneys Potter type I claimed to be specific for the ARPKD, and with microbrachycephaly, hypertelorism with telecanthus, large posteriorly angulated fleshy ears and various congenital malformations including congenital heart defects. We suggest that they represent a previously unrecognized autosomal recessive lethal developmental disorder within the group of infantile polycystic kidney disease and Potter sequence.

Immunocytochemical differential diagnosis of diffuse malignant pleural mesotheliomas--a clinicomorphological study of 158 cases.

Formalin-fixed paraffin-embedded material of 158 diffuse malignant pleural mesotheliomas (DMPMs) was used in order to determine the differential diagnostic value of immunocytochemical probes against 9 different antigens. While vimentin expression was found in only 50% of cases, regardless of their histological subtype all tumours were found to be cytokeratin-positive when an antibody with broad-spectrum cytokeratin reactivity was used. Conversely, none of the cases was immunostained by antisera against carcinoembryonic antigen (CEA), Leu-M1 antigen, chromogranin, S-100 protein, lysozyme and a T-cell associated antigen. The density of inflammatory cell infiltrates reactive with antisera against the three latter antigens was not associated with the clinical behaviour of the neoplasms examined. Eight DMPM cases showed immunoreactivity with HEA-antibodies against Egp 34, an antigen previously supposed only to be expressed by carcinomas. On the basis of these findings, the consistent cytokeratin reactivity, also of the sarcomatous type of DMPM, may help to exclude metastatic involvement of the pleura by a mesenchymal neoplasm of other origin. CEA and Leu-M1 staining of a given pleural tumour, on the other hand, is indicative of a carcinoma secondarily afflicting the pleura, thus making the diagnosis of primary DMPM unlikely.

[Leiomyomatosis of the colon: case report and literature review]. / Leiomyomatose des Rektums: Fallbericht und Literaturübersicht.

We present the findings of a 67 year old male patient with an intestinal leiomyomatosis localized in the rectum. To our knowledge, this is the fifth case of intestinal leiomyomatosis reported so far. The most characteristic findings of this rare disease include a cuff-like tumorous proliferation of smooth muscle within the bowel wall which may extend into extramural tissue and result in a stenosis of a longer bowel segment. Because of severe obstructive symptoms over 5 years, the patient had to undergo surgery with resection of the rectum. The histological examination revealed a morphology and immunophenotype comparable to usual leiomyomas with the exception of hyalinosis-like changes in the blood vessels, apparently a special feature of leiomyomatosis. A novel finding in our case was the occurrence of skeinoid fibers which have so far only been reported in gastrointestinal stromal tumors.

[123I-Metaiodobenzylguanidine- (MIBG-) scintigraphy: paradoxical positivity in an oncocytic adrenocortical carcinoma]. / 123I-Methyljodbenzylguanidin- (MIBG-) Szintigraphie: Paradoxe Nuklidspeicherung eines onkozytären Nebennierenrindenkarzinoms.

(123)I-metaiodobenzylguanidine (MIBG), a radio-labeled catecholamine analogue, is used for the imaging of pheochromocytoma based on the selective uptake of MIBG by chromaffin tissues. MIBG scintigraphy displays high sensitivity (90%) and specificity (close to 100%). In contrast, the false-positive uptake of MIBG by adrenal cortical carcinoma is rare. Here, we report a metastatic oncocytic adrenal cortical carcinoma with MIBG uptake used for therapeutic purposes.

[Diagnostic relevance of multinucleated giant cells in papillary thyroid cancer. A cytological and histological study]. / Diagnostische Relevanz mehrkerniger Riesenzellen beim papillären SchilddrüsenkarzinomEine zytologische und histologische Untersuchung.

Cytological smears of 17 papillary carcinomas (PC) of the thyroid as well as histological slides of 58 PC and 50 follicular adenomas (FA) and 50 follicular carcinomas (FC) were reviewed to assess the presence of intrafollicularly located multinucleated giant cells. In accordance with the data published, such giant cells were found in 70% of PC but in only 8% of FA and FC. The presence of giant cells, which probably represents a foreign body reaction to, in case of PC, physicochemically altered colloid, is a useful additional criteria for the cytological and histological diagnosis of PC.

[Differential diagnosis of destructive processes of the skull base. Aggressive polypoid eosinophilic pansinusitis with expression of carcinoembryonic antigen (CEA)]. / Ein Beitrag zur Differentialdiagnose von destruierenden Prozessen der Schädelbasis. Die aggressive polypöse eosinophile Pansinusitis mit Expression von karzinoembryonalem Antigen (CEA).

We report a woman with extensive polypoid sinusitis and bony destruction of the middle and posterior skull base. Histological examination of the specimen from the paranasal sinuses and adjacent skull base revealed polypoid eosinophilic sinusitis: malignancy could be excluded histologically and clinically. Plasma-CEA-levels were elevated to 85 micrograms/l with no typical source of CEA expression, and they fell after therapy. Immunocytochemistry revealed a remarkable expression of CEA in the surface epithelia of the specimen obtained from the paranasal sinuses. CEA-immunoscintigraphy showed an enhancement in the regions of the paranasal sinuses without expression elsewhere in the body. The authors discuss the possibility of using CEA as a marker for aggressive forms of chronic sinusitis and suggest radiotherapy is a successful treatment option in addition to surgery. The observation period of almost two years confirms the value of the chosen therapy.

Pathology of multiple endocrine neoplasias 2A and 2B: a review.

This review describes recent findings on the morphology, function and prognosis of lesions associated with the MEN 2 syndromes. Special emphasis is placed on the analogies and discrepancies between the hereditary and nonhereditary manifestations of the endocrine proliferations involved.

[Malignant adrenal pheochromocytoma--problems in evaluating clinical diagnosis and morphologic extent]. / Maligne adrenale Phäochromozytome--Probleme der klinischen Diagnostik und morphologischen Dignitätsbestimmung.

Reports on three patients with malignant adrenal phaeochromocytoma are used as a basis for discussion of involved in diagnosing functionally inactive paragangliomas and in discriminating between benign and malignant adrenomedullary tumours. Malignancy can so far only be ascertained in such neoplasms by evidence of metastatic growth. Our findings, however, show that increased tumour weight (more than 200 g), high mitotic activity (more than 5 mitoses per HPF) and loss of S-100 protein-positive subtentacular cells make it possible to distinguish high-risk cases (with increased risk of recurrence and metastasis).

[Morphology, DNA cytophotometry and prognosis in gastrointestinal neuroendocrine tumours (carcinoids). A clinico-pathological study of 95 patients]. / Morphologie, DNA-Zytophotometrie und Prognose gastrointestinaler neuroendokriner Tumoren (Karzinoide). Eine klinisch-pathologische Untersuchung an 95 Patienten.

A total of 123 manifestations (97 primary tumours and 26 metastases) of neuroendocrine tumours of the gastrointestinal tract observed in 95 patients was investigated for the prognostic value of clinical, histological and DNA cytophotometric parameters. Metastases almost exclusively occurred among ileal carcinoids, which also were responsible for all 14 cases of lethal outcome observed during the follow-up period of mean 42 months. Aneuploid DNA values could be determined significantly more frequently among ileal than in non-ileal carcinoids and showed - upon analysis of the total group of gastrointestinal neuroendocrine tumours - a significant correlation to lethal course of disease. In addition, among 18 cases with primary and secondary carcinoid manifestations available for DNA cytophotometry, an association between the DNA content of metastatic neuroendocrine tumours and prognosis came to light. When applied to the group of ileal neoplasms, however, the parameter DNA content did not allow a better prognostic assessment.

[Lymphoepithelial carcinoma of the thyroid gland. A thyroid gland carcinoma with thymus-like differentiation]. / Lymphoepitheliales Karzinom der Schilddrüse. Ein Schilddrüsenkarzinom mit thymusartiger Differenzierung.

An example of lymphoepithelial carcinoma of the thyroid is reported, which was first described 10 years ago and has not as yet been documented in the German literature. This tumour is morphologically identical to the lymphoepithelioma of the nasopharynx. In contrast to neoplasms of such morphology occurring in a variety of other organs, the acronym CASTLE--for carcinoma showing thymus-like differentiation--has been proposed by Chan and Rosai for lymphoepithelioma-like carcinomas of the thyroid gland. In this paper, the aforenamed authors' concept of the histogenesis of thyroid tumours with thymic differentiation is presented.

[Primary hemorrhagic spindle cell tumor of the lymph node with amianthoid structures. Case report and review of the literature]. / Primärer hämorrhagischer Spindelzelltumor des Lymphknotens mit Amianthoidstrukturen. Fallbericht und Literaturübersicht.

An example of a benign haemorrhagic spindle cell tumour of the lymph node (synonym: intranodal myofibroblastoma) is presented, which was first described 5 years ago and has not as yet been documented in the German literature. This rare benign mesenchymal neoplasm of the lymph node occurs almost exclusively in the inguinal region. Diagnostic criteria include its composition of myofibroblastic cells (with immunoreactivity for vimentin and actin), which may show nuclear palisading, the presence of peripheral haemorrhage zones and the occurrence of characteristic amianthoid structures, which have the ultrastructural appearance of dense meshworks of collagen fibers. Knowledge of this tumour entity is important for differential diagnosis of primary and secondary malignant mesenchymal neoplasias of the lymph node.

[IUD-associated pseudoactinomycotic gradiate granules (Spendore-Hoeppli phenomenon) in uterine curettage]. / Pseudoaktinomykotische Granula (Splendore-Hoeppli-Phänomen) im Korpusabradat bei IUP.

Pseudoactinomycotic radiate granules, also known as the Splendore-Hoeppli phenomenon represent a response to organic and in-organic foreign substances and can be found at various locations in the body. They are frequently observed in uterine curettages, where they can be misinterpreted as actinomycotic sulfur granules. Here we report a case with Splendore-Hoeppli phenomenon in an intrauterine device (IUD)-associated genital actinomycosis. The morphological features of this phenomenon are presented using special staining methods and electron microscopy and the literature is reviewed.

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. Immunocyto-chemistry 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 immunostaining 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.

DNA-cytophotometry and immunocytochemistry in ovarian tumours of borderline malignancy and related peritoneal lesions.

A total of 34 surgical specimens, obtained from 13 patients with ovarian tumours of borderline malignancy (OTBM), were investigated by conventional histology, immunocytochemistry and DNA cytophotometry. The lesions were obtained by primary ovarian surgery or second-look procedures and altogether comprised 19 (single and bilateral) OTBM, 8 cases of endosalpingiosis, 4 in situ and 2 invasive peritoneal implants and 1 overt adenocarcinoma. The morphological findings were related to follow-up data, which showed neoplasms with clinically malignant behaviour in 2 patients. The histology of the extra-ovarian manifestations was not associated with their immunocytochemical properties or with their DNA content. There were no correlations between the evolution of disease and microscopical features but the clinical course appeared to be linked to the DNA content of the extra-ovarian lesions, which was of greater prognostic importance than DNA ploidy of the ovarian tumours. Recurrence-free survival was noted in all 5 patients with diploid or euploid extra-ovarian proliferations, while the 2 clinically malignant cases fell into the group of 3 patients with noneuploid or aneuploid specimens. DNA estimations may be a methodology which increases the prognostic value of second-look procedures in OTBM patients.

DNA cytophotometric findings in pheochromocytoma.

Fifty adrenalectomy specimens containing normal (n = 3), hyperplastic (n = 4), or neoplastic (n = 43) medullary tissue were subjected to quantitative measurements of DNA content. Of the 43 pheochromocytomas, 16 were neoplasms inherited in the setting of multiple endocrine neoplasia type 2A. Five of 27 sporadic pheochromocytomas followed a malignant clinical course. Follow-up data were available in 25 patients. In normal medulla and adrenomedullary hyperplasia either diploid or euploid DNA distributions were found. In contrast, 87% (33 of 38) of the benign and all five malignant pheochromocytomas exhibited nondiploid or aneuploid DNA histograms. No differences in DNA content existed between sporadic and hereditary tumors. In contrast to earlier reports, in this study DNA cytophotometry was not suitable to discriminate benign from malignant adrenomedullary tumors. In addition, DNA measurements appeared not to be a useful tool to assess the prognosis of an individual malignant pheochromocytoma.