Evening Specialty Conference - Genitourinary Pathology Case 2 Disclosure of Relevant Financial Relationships Sean R Williamson, MD Henry Ford Health System, Detroit, MI @Williamson_SR USCAP requires that all planners (Education Committee) in a position to influence or control the content of CME disclose any relevant financial relationship WITH COMMERCIAL INTERESTS which they or their spouse/partner have, or have had, within the past 12 months, which relates to the content of this educational activity and creates a conflict of interest. Disclosure of Relevant Financial Relationships USCAP requires that all faculty in a position to influence or control the content of CME disclose any relevant financial relationship WITH COMMERCIAL INTERESTS which they or their spouse/partner have, or have had, within the past 12 months, which relates to the content of this educational activity and creates a conflict of interest. Dr. Williamson declares he has no conflict(s) of interest to disclose. Widespread lymphadenopathy Retroperitoneal Supraclavicular Underwent excisional lymph node biopsy for lymphoma 34 year-old man Supraclavicular lymph node Supraclavicular lymph node 1
Metastatic seminoma Can be challenging in metastatic site limited material or DX not suspected OCT3/4 + D2-40 (podoplanin) KIT Caution: OCT3/4 may be positive in some lymphomas 1. Williams AS, et al. Am J Surg Pathol. 2016;40:950-957. Metastatic seminoma to lung nearly obscured by granulomas Metastatic seminoma to lung nearly obscured by granulomas Metastatic seminoma to lung nearly obscured by granulomas OCT3/4 Metastatic seminoma in retroperitoneal core biopsy 2
Metastatic seminoma in retroperitoneal core biopsy OCT3/4 KIT Patient - Patient - Patient - 3
Patient - May represent spontaneous regression of germ cell tumor Two findings considered specific for regression: GCNIS (formerly IGCNU) Coarse intratubular calcification Balzer BL, Ulbright TM. Am J Surg Pathol. 2006;30:858-865. Scarring in testis Orchiectomy previous biopsy diagnosis of retroperitoneal embryonal carcinoma Coarse calcification in scar Coarse calcification in scar Microlithiasis 4
GCNIS Patient - Patient - Immunohistochemistry PAX8 rete testis PAX8 cyst Some evidence for mixed IHC pattern supporting teratoma Roma A, Varsegi M, Magi-Galluzzi C, et al. The distinction of bronchogenic cyst from metastatic testicular teratoma: a light microscopic and immunohistochemical study. Am J Clin Pathol. 2008;130:265-273. CK7 CK20 CDX2 After chemotherapy, persistent retroperitoneal masses 5
Myogenin After chemotherapy, persistent retroperitoneal masses Somatic type malignancy of germ cell tumor origin Also known as malignant transformation of teratoma or secondary malignant component Often sarcomas RMS > LMS > angio > others PNET Sarcoma NOS to be discussed Criteria for somatic malignancy Overgrowth of 4x magnification field Approx 5 mm diameter Atypia not diagnostic alone Even well-differentiated tissues in teratoma are derived from malignant GCT Often haphazard architecture, atypia Ulbright TM, et al. Cancer. 1984;54:1824-1833. Hypercellular cartilage Atypia in teratoma 6
Ulbright TM, et al. Mod Pathol. 2010;23:972-980. PNET of germ cell tumor origin lacks chr 22 rearrangement (EWSR1) PNET of GCT origin PNET of GCT origin ERMS of GCT origin ERMS of GCT origin Myogenin patchy in embryonal RMS, diffuse in alveolar RMS Treatment - somatic malignancy Surgical resection if at all possible Possibly chemotherapy directed against new histology i.e. sarcoma, etc Traditional platinum GCT tx less likely to be successful 7
Clevenger JA, et al. Mod Pathol. 2009; 22: 1361-1366. Differentiated skeletal muscle in teratoma Absence of mitotic activity, necrosis, primitive cellular component utilized for distinction from embryonal rhabdomyosarcoma Patient liver metastases Patient liver metastases Keratin Howitt BE, et al. Am J Surg Pathol. 2015;39:251-259. 8
Sarcomatoid YST myxoid and fibrous Courtesy of Brooke E. Howitt, MD, Brigham and Womens Hospital Tumor ringlet formation Courtesy of Brooke E. Howitt, MD, Brigham and Womens Hospital GPC3 AE1/AE3 Courtesy of Brooke E. Howitt, MD, Brigham and Womens Hospital Sarcomatoid yolk sac tumor Behaved aggressively when high-grade FNCLCC system (2-3) Likely an alternative pathway to some sarcomas of GCT origin that previously defied classification Howitt BE, et al. Am J Surg Pathol. 2015;39:251-259. Testicular cancer can present with challenging features confounding pathology Metastatic with unknown primary Regression of primary tumor Unusual patterns Transformation to sarcoma or other somatic malignancy Usually post-treatment recurrences Summary #GUPath #USCAP2017 #insitupathologists THANK YOU @Williamson_SR 9