Epithelioid trophoblastic tumor (ETT) is a rare neoplasm derived from chorionic intermediate trophoblast cells, representing less than 2% of all gestational trophoblastic neoplasms. Classically, ETT presents as a uterine mass in women of reproductive age following a term pregnancy. The time from pregnancy to tumor development varies from months to several years. ETT most often arises in the endometrium, followed by the cervix. Extrauterine ETT are extremely infrequent, with few cases reported in the literature. We report a case of a 41-year-old woman, with history of three term pregnancies who presented with abdominal pain and elevated beta human chorionic gonadotropin (β-hCG) level, ten years after her last pregnancy. Imaging reported a 3.5 cm adnexal mass, suspicious for ectopic pregnancy. Hysterectomy and mass resection revealed a 4.7 cm, tan-yellow, necrotic mass adjacent to the broad ligament. Histologic evaluation in conjunction with immunohistochemical stains revealed a tumor consistent with ETT. No connection to the endometrium was found grossly or microscopically. DNA fingerprinting analysis revealed the tumor to have two copies of paternal alleles, as seen in molar gestations. One of the primary differential diagnoses for ETT is squamous cell carcinoma due to similar morphologic features. In challenging cases, genetic analysis demonstrating paternally derived genes can establish the diagnosis. In this report, we discuss the challenges in the diagnosis of extrauterine ETT, due to its rarity and highly variable presentation, given that appropriate diagnosis is critical for correct patient management.
Introduction/Objective Epithelioid trophoblastic tumor (ETT) is an extremely rare neoplasm derived from chorionic type intermediate trophoblast. ETT usually follows an antecedent term pregnancy but can also follow spontaneous abortions or molar pregnancy. ETT most often arises from the endometrium, followed by the cervix. Extrauterine ETT are extremely rare, with few cases reported in literature. Methods/Case Report A 41-year-old woman with three term pregnancies presented with abdominal pain, ten years after her last pregnancy. Imaging findings of a 3.5 cm adnexal mass coupled with an elevated serum β-hCG (~ 900 mIU/ml), led to the suspicion of an ectopic pregnancy. Hysterectomy with salpingectomy revealed a 4.7 cm, tan- yellow, necrotic mass in the adnexal region abutting but distinct from the uterine serosa. Histologic evaluation showed a well- circumscribed tumor with pushing borders. The tumor cells were epithelioid with well-defined eosinophilic cytoplasm, monomorphic nuclei, frequent mitosis, and abundant geographic necrosis. The tumor cells were positive for β-hCG, GATA-3, PLAP and inhibin, with focal weak staining squamous markers p63 and p40. DNA fingerprinting analysis, performed to confirm the diagnosis of ETT, revealed a homozygous tumor with two copies of non-maternal genes indicating that the antecedent index gestation giving rise to the tumor was an undiagnosed hydatidiform mole. Following surgery, serum β-hCG levels were normal and the patient is currently on surveillance. Results (if a Case Study enter NA) NA Conclusion We present an extremely rare case of extrauterine ETT arising from a previously undetected molar pregnancy. The diagnosis should be suspected when a mass is observed at extrauterine sites with elevated β-hCG levels in patients with or without vaginal bleeding. Histologic differential of squamous cell carcinoma needs to be ruled out with immunostains. Due to its rarity and highly variable presentation, this entity remains a diagnostic challenge. DNA fingerprinting analysis demonstrating non-maternal genes can help confirm the diagnosis of ETT.
Introduction: This workshop was created by pathologists' assistants (PAs) who undertake specialized training programs heavily focused on grossing, which is the macroscopic inspection, evaluation, and sampling of pathology specimens for histologic evaluation by a surgical pathologist. Typically, pathologists or their trainees, during training, focus upon microscopic features, with less emphasis on the macroscopic appearance of disease. Methods: We developed monthly hour-long "interesting gross case workshops" (IGCWs) led by PAs to aid in the process of educating and refining pathology residents' grossing skills and abilities to diagnose at a macroscopic level. Our workshops include gross photography, grossing vocabulary, reference to standardized sampling manuals, and viewing of a specimen library to reinforce subject matter. This workshop was designed to train multilevel pathology providers including PA students, residents, fellows, and medical students, in addition to actively practicing PAs. This resource offers the audience preparation materials to host a workshop in any medical training facility. Results: Our use of the IGCWs has been evaluated positively over a 3-year period, with 78 of 150 participants stating they have increased confidence and capabilities to accurately assess and diagnose at the macroscopic level. Discussion: Utilizing this workshop can be valuable to teach the importance of a thorough gross description and tissue sampling for the diagnostic process. Future work includes expansion to additional pathology learners, improving photography database quantity and quality, and incorporating microscopic correlation when necessary.
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