Parachordomas.
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Diagnosis, Differential↗
Biomedical subjects
K W Berean
Publications and source records attributed to K W Berean.
Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma.
Olfactory neuroblastoma (ONB) is a malignant tumor of the nasal mucosa whose histogenesis is unclear. A relationship to neuroblastoma (NB), a pediatric tumor of the sympathetic nervous system, is based on morphologic similarities and the expression of similar neural antigens. However, the clinical presentation of ONB differs from that of NB, and MYCN amplification characteristic of NB is not observed. We have therefore examined the relationship of this malignancy to other classes of neural tumors. In previous studies, two ONB cell lines demonstrated cytogenetic features and patterns of protooncogene expression suggestive of a relationship to the Ewing sarcoma family of childhood peripheral primitive neuroectodermal tumors (pPNETs). The pPNETs show t(11;22)(q24;q12) or t(21;22)(q22;q12) chromosomal translocations fusing the EWS gene from 22q12 with either the FL11 gene on 11q24 or the ERG gene on 21q22. We therefore analyzed ONBs for the presence of pPNET-associated gene fusions. Both cell lines showed rearrangement of the EWS gene, and fluorescence in situ hybridization (FISH) of each case demonstrated fusion of EWS and FL11 genomic sequences. Moreover, both lines expressed EWS/FL11 fusion transcripts with in-frame junctions between exon 7 of EWS and exon 6 of FL11 as described for pPNETs. We identified similar gene fusions in four of six primary ONB cases. None of the cases expressed tyrosine hydroxylase, a catecholamine biosynthetic enzyme widely expressed in NB. Our studies indicate that ONB is not a NB but is a member of the pPNET family.
Adult↗
Quantitative image cytometry of infiltrating ductal carcinoma: comparison with prognostic parameters and reproducibility of histological grade.
Quantitative image cytometry was used to compare 18 parameters relating to ploidy, nuclear area, and chromatin texture to axillary lymph node status, tumor size, and histological grade for 34 infiltrating ductal carcinomas, each of which had been graded independently by each of six surgical pathologists. Zinc formalin-fixed, paraffinembedded tumors were assessed using the Elston and Ellis modification of the Bloom and Richardson histological grading scheme. When axillary lymph node-negative tumors were compared with those involving four or more nodes, % 2 c (diploid) cells, nuclear area, and eight of 12 chromatin texture parameters showed statistically significant differences. Carcinomas < 2 cm had more % 2 c (diploid) cells and fewer % > 4 c (hypertetraploid) cells than larger neoplasms. For tumors having nuclear pleomorphism score two versus those with score three, nuclear area, four of five parameters related to ploidy level, each of five parameters related to run-length matrix features and one of four co-occurrence matrix features showed significant differences. Nearly all of these cytometric parameters also showed significant differences for histological grade and mitotic count, which was strongly correlated with nuclear pleomorphism. In examining the cytometric parameters in relation to the interobserver reproducibility of histological grade and its components, the largest number of statistically significant parameters related to the nonreproducibility of nuclear pleomorphism. The findings indicate that as the grade of infiltrating ductal carcinomas increases, there are fewer % 2 c (diploid) cells and more % > 4 c (hypertetraploid) and % > or = 5 c (polyploid) cells. In addition, the cells of high grade tumors have larger nuclear areas and more small and large dense chromatin clumps, which increase in such number that they tend to join together. When compared with the cytometric parameters, nuclear pleomorphism is the most sensitive component of grade to nonreproducibility.
Breast Neoplasms↗
Primary ductal adenocarcinoma of the lacrimal gland.
PURPOSE: To their knowledge, the authors report the first recognized case of ductal adenocarcinoma of the lacrimal gland (histologic equivalent of salivary duct carcinoma). Primary adenocarcinoma of the lacrimal gland is rare and has been described generically. In contrast, primary adenocarcinomas of the major and minor salivary glands are much more common and have been classified into histopathologic subtypes that have different clinical characteristics and outcomes. METHODS: A 68-year-old man presented with a 6-month history of a painless mass in the right upper outer eyelid. The authors describe the clinical, radiologic, and histopathologic features of this case and review the lacrimal gland literature. RESULTS: A modified en bloc orbitectomy was performed, and postoperative radiotherapy was administered. The patient was alive and well without evidence of tumor recurrence 10 months after surgery. CONCLUSION: The World Health Organization classification of salivary adenocarcinomas (1991) provides a framework for further insight into the presentation and biologic behavior of the less common lacrimal carcinomas.
Aged↗
Salivary duct carcinoma secreting prostate-specific antigen.
Prostate-specific antigen (PSA) is a 30 kDa glycoprotein serine protease that shows high tissue specificity for prostatic tissue, both benign and malignant. However, recent reports have shown that a variety of normal and neoplastic tissue types express PSA immunohistochemically. In addition, rare instances of the secretion of PSA by nonprostatic cancers have been reported in the literature. The authors present a case of salivary duct carcinoma associated with elevated serum levels of PSA. Both the primary tumor and metastases stained positively with anti-PSA monoclonal antibodies, but were negative with antibodies directed against prostate-specific acid phosphatase. Elevated serum PSA levels were confirmed with three different immunoassay methods. A peak serum level of 140 micrograms/L was measured and this correlates with levels of PSA associated with metastatic prostatic carcinoma. High performance liquid chromatography with a molecular sieve column characterized the serum PSA into both free protein (approximately 20%) and protein bound to alpha-1-antichymotrypsin (PSA-ACT)(approximately 80%). Molecular weights of the free PSA and PSA-ACT subfractions were 27-31 kDa and 100-110 kDa, respectively.
Adenocarcinoma↗
Intraneural biphasic synovial sarcoma: an alternative "glandular" tumor of peripheral nerve.
A primary intraneural biphasic synovial sarcoma that arose within the common digital nerve of the hand is reported. The patient, a 16-year-old girl, presented with a small soft tissue mass that clinically and intraoperatively resembled a nerve sheath tumor. The diagnosis of synovial sarcoma was established on the basis of a typical light microscopic appearance and immunohistochemical staining profile. Intraneural biphasic synovial sarcoma resembles "glandular" peripheral nerve sheath tumor, because both are composed of a mixture of spindled mesenchymal cells with admixed glandular epithelial elements. The features allowing differentiation of these two unusual tumors of peripheral nerve are discussed.
Adolescent↗
Pathologic quiz case 2. Cherubism.
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Adult↗
Interobserver reproducibility of the Nottingham modification of the Bloom and Richardson histologic grading scheme for infiltrating ductal carcinoma.
The interobserver reproducibility of the Nottingham modification of the Bloom and Richardson histologic grading scheme for invasive breast carcinoma was tested. Six surgical pathologists from four institutions independently evaluated histologic grade and each of its three components for 75 infiltrating ductal carcinomas. The number of slides per case ranged from one to nine (median 3). Pairwise kappa values for agreement ranged from moderate to substantial (0.43-0.74) for histologic grade. Generalized kappa values indicated substantial agreement for tubule formation (0.64), moderate agreement for mitotic count (0.52), and near moderate agreement for nuclear pleomorphism (0.40). Normalizing the mitotic counts per mm2 showed only slight improvement in agreement over the published range of mitotic counts for three different field areas. The results suggest that steps to discriminate between categories for nuclear pleomorphism would likely be of benefit for improving the interobserver reproducibility of histologic grade. Nevertheless, the Nottingham modification of the Bloom and Richardson grading system is recommended as a suitable scheme for evaluating invasive breast carcinomas in the routine clinical setting.
Breast Neoplasms↗
Multiple oncocytic laryngeal cysts presenting as acute airway obstruction.
Solitary oncocytic cyst of the larynx is a recognized clinical and pathologic entity. Multiple oncocytic cysts have only rarely been described. A 67-year-old female presented with rapidly progressive airway obstruction requiring emergency tracheotomy. The obstruction was caused by multiple cystic lesions throughout the supraglottic region. Microscopic examination of laryngeal biopsies showed cysts within the lamina propria, lined by oncocytic epithelium. The differential diagnosis and pathogenesis of oncocytic cysts is discussed with a review of other laryngeal cystic lesions.
Aged↗
Differentiation of thyroid neoplasms by evaluating epithelial membrane antigen, Leu-7 antigen, epidermal growth factor receptor, and DNA content.
The expression of epithelial membrane antigen (EMA), Leu-7 antigen, epidermal growth factor receptor (EGFr), and deoxyribonucleic acid (DNA) content in 40 thyroid nodules was investigated to identify those factors that might differentiate these lesions or correlate to their prognosis. There were 22 carcinomas (16 papillary, 4 follicular, 1 anaplastic, 1 medullary) and 18 benign lesions prospectively obtained between 1989 and 1993. Patients' charts were reviewed to establish a database of known clinical prognostic indicators. Expression of EMA and Leu-7 was significant in malignant lesions when compared to benign lesions (P < 0.02 and P < 0.001). EMA was expressed significantly more frequently by follicular carcinomas than by follicular adenomas (P < 0.03). Leu-7 antigen was expressed by all papillary carcinomas. Neither of these antigens showed any association with known clinical prognostic indicators. EGFr expression neither differentiated benign from malignant lesions nor correlated with prognostic factors. The presence of aneuploidy correlated with poor tumor differentiation (P < 0.02), but did not distinguish benign from malignant lesions. These results suggest that EMA expression may be useful for confirming malignancy in follicular neoplasms and that the expression of Leu-7 antigen can assist in distinguishing papillary carcinoma from benign lesions with pseudopapillae.
Adolescent↗