A Agaimy


Affiliation: University of Erlangen-Nuremberg
Country: Germany


  1. Agaimy A. Paraneoplastic disorders associated with miscellaneous neoplasms with focus on selected soft tissue and Undifferentiated/ rhabdoid malignancies. Semin Diagn Pathol. 2019;: pubmed publisher
  2. Agaimy A. SWI/SNF Complex-Deficient Soft Tissue Neoplasms: A Pattern-Based Approach to Diagnosis and Differential Diagnosis. Surg Pathol Clin. 2019;12:149-163 pubmed publisher
    ..This review summarizes and discusses the morphologic and phenotypic diversity of primary soft tissue neoplasms characterized by SWI/SNF complex deficiency with an emphasis on convergent and divergent cytoarchitectural patterns. ..
  3. Agaimy A, Schneider Stock R. [Gastrointestinal stromal tumors: evolution of a tumor concept from unclassifiable neoplasms to targeted molecular therapy]. Pathologe. 2010;31:115-22 pubmed publisher
    ..This article gives an overview on current developments in the histopathological and molecular diagnostics of GIST and the role of kinase mutation analysis for optimizing patient treatment. ..
  4. Ohlmann C, Brecht I, Junker K, van der Zee J, Nistor A, Bohle R, et al. Sclerosing epithelioid fibrosarcoma of the kidney: clinicopathologic and molecular study of a rare neoplasm at a novel location. Ann Diagn Pathol. 2015;19:221-5 pubmed publisher
    ..Awareness of this entity would help avoid misinterpretation as clear cell carcinoma, sclerosing perivascular epithelioid cell tumor, Xp.11 translocation carcinoma, and other more frequent neoplasms at this site. ..
  5. Agaimy A, Hartmann A. [Hereditary and non-hereditary syndromic gastointestinal stromal tumours]. Pathologe. 2010;31:430-7 pubmed publisher
    ..This review summarizes the pertinent clinicopathological and molecular features of the main GIST syndromes to facilitate their diagnosis and distinction from their non-syndromic mimics. ..
  6. Erber R, Agaimy A. Misses and near misses in diagnosing nodular fasciitis and morphologically related reactive myofibroblastic proliferations: experience of a referral center with emphasis on frequency of USP6 gene rearrangements. Virchows Arch. 2018;473:351-360 pubmed publisher
  7. Erlenbach Wünsch K, Bihl M, Hartmann A, Groisman G, Vieth M, Agaimy A. Serrated epithelial colorectal polyps (hyperplastic polyps, sessile serrated adenomas) with perineurial stroma: Clinicopathological and molecular analysis of a new series. Ann Diagn Pathol. 2018;35:48-52 pubmed publisher
    ..A more concise terminology reflecting their epithelial nature is needed to fulfill the requirements for colorectal cancer risk assessment and hence adopt appropriate follow-up strategies. ..
  8. Agaimy A, Mudter J, Märkl B, Chetty R. Cytomegalovirus infection presenting as isolated inflammatory polyps of the gastrointestinal tract. Pathology. 2011;43:440-6 pubmed publisher
    ..Awareness of these unusual lesions should enhance detection and proper classification of this probably under-recognised CMV presentation. ..
  9. Agaimy A, Ben Izhak O, Lorey T, Scharpf M, Rubin B. Angiosarcoma arising in association with vascular Dacron grafts and orthopedic joint prostheses: clinicopathologic, immunohistochemical, and molecular study. Ann Diagn Pathol. 2016;21:21-8 pubmed publisher
    ..Limited awareness of their existence explains delayed clinical diagnosis in most of cases. Absence of MDM2/CDK4 alterations underlines their distinction from intimal-type sarcomas. ..

More Information


  1. Kramer K, Wolf S, Mayer B, Schmidt S, Agaimy A, Henne Bruns D, et al. Frequence, spectrum and prognostic impact of additional malignancies in patients with gastrointestinal stromal tumors. Neoplasia. 2015;17:134-40 pubmed publisher
    ..Moreover the data indicate a strong impact of additional malignant neoplasms in GIST on patient outcome. A comprehensive follow-up strategy of GIST patients appears to be warranted. ..
  2. Agaimy A, Michal M, Thompson L, Michal M. Angioleiomyoma of the Sinonasal Tract: Analysis of 16 Cases and Review of the Literature. Head Neck Pathol. 2015;9:463-73 pubmed publisher
    ..Submucosal sinonasal ALMs are rare benign tumors similar to their reported cutaneous counterparts with frequent adipocytic differentiation. They should be distinguished from renal-type angiomyolipoma. Simple excision is curative. ..
  3. Agaimy A, Carney J. Lymphatics and D2-40/podoplanin expression in gastrointestinal stromal tumours of the stomach with and without lymph node metastasis: an immunohistochemical study with special reference to the Carney triad. J Clin Pathol. 2010;63:229-34 pubmed publisher
    ..The biological significance of D2-40/podoplanin expression in tumour cells of syndromic/paediatric GISTs and its possible role in lymphatic tumour spread remain to be further investigated. ..
  4. Agaimy A, Märkl B. Inflammatory angiomyolipoma of the liver: an unusual case suggesting relationship to IgG4-related pseudotumor. Int J Clin Exp Pathol. 2013;6:771-9 pubmed
    ..A possible role for IgG4 seems likely to explain the peculiar histological features and the unusual clinical presentation in this case. ..
  5. Agaimy A, Thiel F, Hartmann A, Fukunaga M. SMARCA4-deficient undifferentiated carcinoma of the ovary (small cell carcinoma, hypercalcemic type): clinicopathologic and immunohistochemical study of 3 cases. Ann Diagn Pathol. 2015;19:283-7 pubmed publisher
  6. Strehl J, Wachter D, Fiedler J, Heimerl E, Beckmann M, Hartmann A, et al. Pattern of SMARCB1 (INI1) and SMARCA4 (BRG1) in poorly differentiated endometrioid adenocarcinoma of the uterus: analysis of a series with emphasis on a novel SMARCA4-deficient dedifferentiated rhabdoid variant. Ann Diagn Pathol. 2015;19:198-202 pubmed publisher
    ..This case further underlines the close link between the "rhabdoid phenotype" and the SWI/SNF pathway. ..
  7. Agaimy A. [Value of core needle biopsy in preoperative diagnostics of soft tissue tumors: possibilities and limitations]. Pathologe. 2014;35 Suppl 2:189-97 pubmed publisher
    ..The utilization of modern immunohistochemistry and/or FISH methods is highly useful for establishing the diagnosis of rare and unusual neoplasms in core needle biopsies. ..
  8. Agaimy A, Amin M, Gill A, Popp B, Reis A, Berney D, et al. SWI/SNF protein expression status in fumarate hydratase-deficient renal cell carcinoma: immunohistochemical analysis of 32 tumors from 28 patients. Hum Pathol. 2018;77:139-146 pubmed publisher
  9. Agaimy A, Michal M, Hadravsky L, Michal M. Dedifferentiated liposarcoma composed predominantly of rhabdoid/epithelioid cells: a frequently misdiagnosed highly aggressive variant. Hum Pathol. 2018;77:20-27 pubmed publisher
    ..This highly aggressive liposarcoma variant needs to be distinguished from a variety of neoplasms including undifferentiated carcinoma, melanoma, lymphoma, rhabdomyosarcoma and others. ..
  10. Agaimy A, Weichert W, Haller F, Hartmann A. [Diagnostic and predictive molecular pathology of head and neck neoplasms]. Pathologe. 2018;39:42-47 pubmed publisher
    ..In the present review we summarize the relevant molecular alterations in topographically and histopathologically distinct tumors of the head and neck region with emphasis on recently established molecular markers. ..
  11. Agaimy A. [Succinate dehydrogenase (SDH)-deficient renal cell carcinoma]. Pathologe. 2016;37:144-52 pubmed publisher
    ..In this review, the major clinicopathological, immunohistochemical and genetic features of this rare disease entity are presented and discussed in the context of the broad differential diagnosis. ..
  12. Agaimy A, Weichert W. [Grading of head and neck neoplasms]. Pathologe. 2016;37:285-92 pubmed publisher
    ..In this article the most important grading schemes and criteria for head and neck malignancies are presented and their prognostic and therapeutic implications are discussed. ..
  13. Agaimy A, Semrau S, Koch M, Thompson L. Sinonasal Leiomyosarcoma: Clinicopathological Analysis of Nine Cases with Emphasis on Common Association with Other Malignancies and Late Distant Metastasis. Head Neck Pathol. 2018;12:463-470 pubmed publisher
    ..Their varied morphology underlines the wide differential diagnostic considerations. Long-term survival may be achieved with aggressive multimodal therapy. ..
  14. Siegler L, Erber R, Burghaus S, Brodkorb T, Wachter D, Wilkinson N, et al. Fumarate hydratase (FH) deficiency in uterine leiomyomas: recognition by histological features versus blind immunoscreening. Virchows Arch. 2018;472:789-796 pubmed publisher
    ..Our data showed predictive morphology to be superior to blind IHC screening for detecting them. The relationship of FH-deficient uterine smooth muscle tumors to the HLRCC syndrome needs further clarification. ..
  15. Agaimy A, Michal M. Hybrid schwannoma-perineurioma of the gastrointestinal tract: a clinicopathologic study of 2 cases and reappraisal of perineurial cells in gastrointestinal schwannomas. Appl Immunohistochem Mol Morphol. 2011;19:454-9 pubmed publisher
    ..We conclude that hybrid schwannoma-perineuriomas are distinct from gastrointestinal schwannoma, both histologically and immunohistochemically. ..
  16. Agaimy A, Haller F, Hartmann A. [Sinonasal tumors : News from the WHO with special reference to mesenchymal entities]. Pathologe. 2018;39:18-26 pubmed publisher
    ..This review summarizes the new aspects in the WHO classification and also addresses recently described entities that have not been included in the WHO classification. ..
  17. Agaimy A, Michal M, Hadravsky L, Michal M. Follicular dendritic cell sarcoma: clinicopathologic study of 15 cases with emphasis on novel expression of MDM2, somatostatin receptor 2A, and PD-L1. Ann Diagn Pathol. 2016;23:21-8 pubmed publisher
    ..FDCS occurring within the retroperitoneum and/or the abdominal cavity may closely mimic dedifferentiated liposarcoma, particularly if MDM2 positive and/or amplified and should thus be carefully assessed for expression of FDC markers. ..
  18. Agaimy A, Hartmann A. [Mesenchymal tumors of the urinary bladder]. Pathologe. 2016;37:61-70 pubmed publisher
    ..This overview summarizes the clinicopathological and differential diagnostic aspects of the most important lesions and discuss their differential diagnosis in the light of current knowledge. ..
  19. Söder S, Sesselmann S, Aigner T, Oehler S, Agaimy A. Tenosynovial giant cell tumour (pigmented villonodular synovitis-)-like changes in periprosthetic interface membranes. Virchows Arch. 2016;468:231-8 pubmed publisher
    ..Although TSGCT and TSGCT-like periprosthetic membranes represent different entities, their comparable morphology might reflect analogous morphogenesis. ..
  20. Agaimy A. Gastrointestinal stromal tumors (GIST) from risk stratification systems to the new TNM proposal: more questions than answers? A review emphasizing the need for a standardized GIST reporting. Int J Clin Exp Pathol. 2010;3:461-71 pubmed
  21. Agaimy A, Mueller S, Harrer T, Bauer S, Thompson L. Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases. Head Neck Pathol. 2018;12:511-516 pubmed publisher
    ..KS should be included in the differential diagnosis of difficult-to-classify spindle cell lesions at this uncommon location. ..
  22. Agaimy A, Bertz S, Cheng L, Hes O, Junker K, Keck B, et al. Loss of expression of the SWI/SNF complex is a frequent event in undifferentiated/dedifferentiated urothelial carcinoma of the urinary tract. Virchows Arch. 2016;469:321-30 pubmed publisher
  23. Agaimy A, Michal M, Giedl J, Hadravsky L, Michal M. Superficial acral fibromyxoma: clinicopathological, immunohistochemical, and molecular study of 11 cases highlighting frequent Rb1 loss/deletions. Hum Pathol. 2017;60:192-198 pubmed publisher
    ..Our results showed frequent Rb1 deficiency as a possible driver molecular event in SAF (seen in 90% of cases) indicating relationship of SAF to the RB1-deleted tumor family. ..
  24. Herpel E, Rieker R, Dienemann H, Muley T, Meister M, Hartmann A, et al. SMARCA4 and SMARCA2 deficiency in non-small cell lung cancer: immunohistochemical survey of 316 consecutive specimens. Ann Diagn Pathol. 2017;26:47-51 pubmed publisher
  25. Agaimy A, Foulkes W. Hereditary SWI/SNF complex deficiency syndromes. Semin Diagn Pathol. 2018;35:193-198 pubmed publisher
    ..This review summarizes the most important SWI/SNF-driven diseases with a main focus on neoplasms. ..
  26. Agaimy A, Hartmann A. Uncovering Hereditary Tumor Syndromes: Emerging Role of Surgical Pathology. Semin Diagn Pathol. 2018;35:154-160 pubmed publisher
    ..The details of some of these entities are in addition dealt with in reviews devoted to them in this special issue. ..
  27. Kramer K, Knippschild U, Mayer B, Bögelspacher K, Spatz H, Henne Bruns D, et al. Impact of age and gender on tumor related prognosis in gastrointestinal stromal tumors (GIST). BMC Cancer. 2015;15:57 pubmed publisher
    ..Extended studies are warranted to confirm our clinical results and to elucidate underlying pathophysiological mechanisms. ..
  28. Agaimy A, Weichert W. SMARCA4-deficient Sinonasal Carcinoma. Head Neck Pathol. 2017;11:541-545 pubmed publisher
  29. Agaimy A, Stoehr R, Vieth M, Hartmann A. Benign serrated colorectal fibroblastic polyps/intramucosal perineuriomas are true mixed epithelial-stromal polyps (hybrid hyperplastic polyp/mucosal perineurioma) with frequent BRAF mutations. Am J Surg Pathol. 2010;34:1663-71 pubmed publisher
    ..The perineurial stromal component might be derived from modified pericryptic fibroblasts as a consequence of a yet poorly understood epithelial-stromal interaction...
  30. Agaimy A, Ihrler S, Märkl B, Lell M, Zenk J, Hartmann A, et al. Lipomatous salivary gland tumors: a series of 31 cases spanning their morphologic spectrum with emphasis on sialolipoma and oncocytic lipoadenoma. Am J Surg Pathol. 2013;37:128-37 pubmed publisher
    ..Oncocytic lipoadenoma is an epithelial-predominant tumor that is distinct from the fat-dominated nononcocytic sialolipoma, but the 2 types seem to merge in some cases...
  31. Agaimy A, Märkl B, Kitz J, Wünsch P, Arnholdt H, Fuzesi L, et al. Peripheral nerve sheath tumors of the gastrointestinal tract: a multicenter study of 58 patients including NF1-associated gastric schwannoma and unusual morphologic variants. Virchows Arch. 2010;456:411-22 pubmed publisher
    ..Gastrointestinal PNSTs are still underrecognized by general pathologists. Awareness of their diverse morphology will help to avoid confusing them with smooth muscle neoplasms and GIST that they may closely mimic...
  32. Agaimy A, Terracciano L, Dirnhofer S, Tornillo L, Foerster A, Hartmann A, et al. V600E BRAF mutations are alternative early molecular events in a subset of KIT/PDGFRA wild-type gastrointestinal stromal tumours. J Clin Pathol. 2009;62:613-6 pubmed publisher
    ..Results from this and a previous study indicate that BRAF-mutated GISTs show a predilection for the small bowel (four of five tumours), but this needs further evaluation in larger studies. ..
  33. Agaimy A, Haller F, Gunawan B, Wünsch P, Fuzesi L. Distinct biphasic histomorphological pattern in gastrointestinal stromal tumours (GISTs) with common primary mutations but divergent molecular cytogenetic progression. Histopathology. 2009;54:295-302 pubmed publisher
    ..Our results indicate that the intratumoral phenotypic diversity in GIST reflects histomorphological progression, which is associated with higher chromosomal instability, irrespective of the primary mutation. ..
  34. Agaimy A, Strecker T. Left atrial myxoma with papillary fibroelastoma-like features. Int J Clin Exp Pathol. 2011;4:307-11 pubmed
    ..This unusual case may shed light on the pathogenesis of the PFE pattern...
  35. Agaimy A. Anisometric cell lipoma: Insight from a case series and review of the literature on adipocytic neoplasms in survivors of retinoblastoma suggest a role for RB1 loss and possible relationship to fat-predominant ("fat-only") spindle cell lipoma. Ann Diagn Pathol. 2017;29:52-56 pubmed publisher
    ..Thus, it seems that Somatic RB1 loss probably drives sporadic ACL in a comparable way to post-retinoblastoma lipomas which were shown to be driven by LOH of the RB1 wild-type allele. ..
  36. Agaimy A, Fuchs F, Moskalev E, Sirbu H, Hartmann A, Haller F. SMARCA4-deficient pulmonary adenocarcinoma: clinicopathological, immunohistochemical, and molecular characteristics of a novel aggressive neoplasm with a consistent TTF1neg/CK7pos/HepPar-1pos immunophenotype. Virchows Arch. 2017;471:599-609 pubmed publisher
    ..Uniform HepPar-1 expression in this subset of NSCLC may represent a diagnostic pitfall and merits further studies to explore the mechanisms involved. ..
  37. Agaimy A. [Soft tissue tumors in hereditary tumor syndromes]. Pathologe. 2017;38:170-178 pubmed publisher
    ..This review summarizes the main syndromes and their associated soft tissue tumors and discusses their characteristic pathological features that help in the recognition of hereditary syndromes. ..
  38. Giedl J, Büttner Herold M, Wach S, Wullich B, Hartmann A, Agaimy A. Hepatocyte differentiation markers in adenocarcinoma of the prostate: hepatocyte paraffin 1 but not arginase-1 is specifically expressed in a subset of prostatic adenocarcinoma. Hum Pathol. 2016;55:101-7 pubmed publisher
    ..18%), highlighting the value of Arg-1 in distinguishing HepPar-1-positive prostatic carcinoma from HCC at metastatic sites or in cases of liver metastasis from prostate carcinoma. ..
  39. Schwarz S, Müller M, Ettl T, Stockmann P, Zenk J, Agaimy A. Morphological heterogeneity of oral salivary gland carcinomas: a clinicopathologic study of 41 cases with long term follow-up emphasizing the overlapping spectrum of adenoid cystic carcinoma and polymorphous low-grade adenocarcinoma. Int J Clin Exp Pathol. 2011;4:336-48 pubmed
    ..Our results emphasize the diversity of oral salivary gland carcinomas and the overlapping clinicopathological features of ACC and PLGA...
  40. Agaimy A, Wünsch P, Hofstadter F, Schroeder J. Hyaline globules in paucicellular leiomyomas of the gastrointestinal tract are distinct from skeinoid fibers and represent degenerating smooth muscle cells. Pathol Res Pract. 2009;205:417-22 pubmed publisher
    ..Awareness of this finding would prevent misinterpretation as GIST, particularly in small biopsies. ..
  41. Agaimy A, Vassos N, Wünsch P, Hohenberger W, Hartmann A, Croner R. Impact of serosal involvement/extramural growth on the risk of synchronous and metachronous peritoneal spread in gastrointestinal stromal tumors: proposal for a macroscopic classification of GIST. Int J Clin Exp Pathol. 2012;5:12-22 pubmed
  42. Agaimy A, Vassos N, Croner R. Gastrointestinal manifestations of neurofibromatosis type 1 (Recklinghausen's disease): clinicopathological spectrum with pathogenetic considerations. Int J Clin Exp Pathol. 2012;5:852-62 pubmed
    ..In addition, this review underlines the great value of specific gastrointestinal findings in uncovering undiagnosed or missed NF-1 cases...
  43. Agaimy A, Wang L, Eck M, Haller F, Chetty R. Loss of DOG-1 expression associated with shift from spindled to epithelioid morphology in gastric gastrointestinal stromal tumors with KIT and platelet-derived growth factor receptor ? mutations. Ann Diagn Pathol. 2013;17:187-91 pubmed publisher
    ..These cases confirm the immunophenotypic heterogeneity as secondary events in GIST. Loss of DOG-1 in KIT-negative PDGFRA mutants should not preclude diagnosis. ..
  44. Agaimy A, Iro H, Zenk J. [Pediatric salivary gland tumors and tumor-like lesions]. Pathologe. 2017;38:294-302 pubmed publisher
    ..Diagnosis is mainly based on a set of typical clinical and imaging features, serological/microbiological findings and, in selected cases, histomorphological characteristics in biopsy specimens. ..
  45. Agarwal J, Kadakia S, Agaimy A, Ogadzanov A, Khorsandi A, Chai R. Pleomorphic liposarcoma of the head and neck: Presentation of two cases and literature review. Am J Otolaryngol. 2017;38:505-507 pubmed publisher
    ..We present the first case series of PL of the head and neck in the literature. ..