Source:http://linkedlifedata.com/resource/pubmed/id/19194280
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions |
umls-concept:C0006826,
umls-concept:C0010606,
umls-concept:C0205422,
umls-concept:C0332284,
umls-concept:C0334347,
umls-concept:C0542559,
umls-concept:C0868928,
umls-concept:C1305968,
umls-concept:C1527178,
umls-concept:C1705938,
umls-concept:C1857941,
umls-concept:C1880371,
umls-concept:C2347662,
umls-concept:C2603343
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| pubmed:issue |
5
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| pubmed:dateCreated |
2009-4-27
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| pubmed:abstractText |
The authors present a series of 24 malignant neoplasms arising in preexisting benign spiradenoma (20), cylindroma (2), and spiradenocylindroma (2). Nineteen patients (12 females, 7 males; age range, 41 to 92 y) had a solitary neoplasm (size range, 2.2 to 17.5 cm; median 4 cm), whereas the remaining 5 (4 females, 1 male; age range, 66 to 72 y) manifested clinical features of Brooke-Spiegler syndrome (BSS), an autosomal dominantly inherited disease characterized by widespread, small, benign neoplasms on which background larger malignant lesions appeared. Microscopically, all cases showed the residuum of a preexisting benign neoplasm. The malignant components of the lesions were variable and could be classified into 4 main patterns, occurring alone or in combination: 1) salivary gland type basal cell adenocarcinoma-like pattern, low-grade (BCAC-LG); 2) salivary gland type basal cell adenocarcinoma-like pattern, high-grade (BCAC-HG); 3) invasive adenocarcinoma, not otherwise specified (IAC-NOS); and 4) sarcomatoid (metaplastic) carcinoma. In 1 case of IAC-NOS, an in situ adenocarcinoma was also found, presumed to have evolved from an adjacent adenomatous and atypical adenomatous component. Cases harboring a sarcomatoid carcinoma featured a malignant epithelial component composed of varying combinations of BCAC-HG, BCAC-LG, IAC-NOS, or squamous cell carcinoma, whereas the sarcomatoid component appeared as either a pleomorphic or spindle-cell sarcoma. Additionally, in 2 cases there were foci of heterologous chondrosarcomatous differentiation and in 1 case there was rhabomyosarcomatous differentiation. Of the 21 patients with available follow-up (range, 3 mo-15 y; average 4.8 y; median 3.5 y), 10 were without evidence of disease, 1 was alive with metastatic disease, 1 was alive with BSS, 3 developed local recurrences, 4 had died of disease, and 2 were dead of other causes. The histologic pattern of the malignant neoplasm correlated to some extent with the clinical course. BCAC-LG neoplasms showed a less aggressive course, with local recurrences but no distant metastases, whereas the BCAC-HG neoplasms typically followed a highly aggressive course resulting in the death 3 of 6 patients with BCAC-HG. Patients with sarcomatoid carcinoma had a relatively good survival. Molecular genetic investigations revealed no mutations in the CYLD gene in the 4 sporadic cases investigated. One patient with BSS revealed a novel missense germline mutation in exon 14 (c. 1961T>A, p. V654E), whereas a living descendant of another deceased patient demonstrated a recurrent nonsense germline mutation in exon 20 (c. 2806C>T, p. R936X). Given the morphologic diversity and complexity of the neoplasms in question, we propose using a more specific terminology with the precise description of the neoplasm components, rather than generic and less informative terms such as "spiradenocarcinoma" or "carcinoma ex cylindroma."
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| pubmed:language |
eng
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| pubmed:journal | |
| pubmed:citationSubset |
IM
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| pubmed:chemical | |
| pubmed:status |
MEDLINE
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| pubmed:month |
May
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| pubmed:issn |
1532-0979
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| pubmed:author |
pubmed-author:CalonjeEduardoE,
pubmed-author:DanisDusanD,
pubmed-author:GraysonWayneW,
pubmed-author:GrossmannPetrP,
pubmed-author:KacerovskaDenisaD,
pubmed-author:KazakovDmitry VDV,
pubmed-author:KonanP GPG,
pubmed-author:MukensnablPetrP,
pubmed-author:RüttenArnoA,
pubmed-author:SimaRadekR,
pubmed-author:SpagnoloDominic VDV,
pubmed-author:VanecekTomasT,
pubmed-author:VazmitelMarinaM,
pubmed-author:XXX,
pubmed-author:ZelgerBernhardB
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| pubmed:issnType |
Electronic
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| pubmed:volume |
33
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| pubmed:owner |
NLM
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| pubmed:authorsComplete |
Y
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| pubmed:pagination |
705-19
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| pubmed:meshHeading |
pubmed-meshheading:19194280-Adenocarcinoma,
pubmed-meshheading:19194280-Adenoma,
pubmed-meshheading:19194280-Adult,
pubmed-meshheading:19194280-Aged,
pubmed-meshheading:19194280-Aged, 80 and over,
pubmed-meshheading:19194280-Australia,
pubmed-meshheading:19194280-Carcinoma,
pubmed-meshheading:19194280-Carcinoma, Adenoid Cystic,
pubmed-meshheading:19194280-Carcinoma, Skin Appendage,
pubmed-meshheading:19194280-Carcinoma, Squamous Cell,
pubmed-meshheading:19194280-Cell Differentiation,
pubmed-meshheading:19194280-Chromosomes, Human, Pair 16,
pubmed-meshheading:19194280-Europe,
pubmed-meshheading:19194280-Female,
pubmed-meshheading:19194280-Gene Expression Regulation, Neoplastic,
pubmed-meshheading:19194280-Humans,
pubmed-meshheading:19194280-Immunohistochemistry,
pubmed-meshheading:19194280-Male,
pubmed-meshheading:19194280-Metaplasia,
pubmed-meshheading:19194280-Middle Aged,
pubmed-meshheading:19194280-Mutation,
pubmed-meshheading:19194280-Neoplasm Invasiveness,
pubmed-meshheading:19194280-Neoplasms, Multiple Primary,
pubmed-meshheading:19194280-Salivary Gland Neoplasms,
pubmed-meshheading:19194280-Sarcoma,
pubmed-meshheading:19194280-Soft Tissue Neoplasms,
pubmed-meshheading:19194280-South Africa,
pubmed-meshheading:19194280-Syndrome,
pubmed-meshheading:19194280-Treatment Outcome,
pubmed-meshheading:19194280-Tumor Suppressor Proteins
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| pubmed:year |
2009
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| pubmed:articleTitle |
Morphologic diversity of malignant neoplasms arising in preexisting spiradenoma, cylindroma, and spiradenocylindroma based on the study of 24 cases, sporadic or occurring in the setting of Brooke-Spiegler syndrome.
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| pubmed:affiliation |
Sikl's Department of Pathology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic. kazakov@medima.cz
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| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't,
Multicenter Study
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