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Weekly Chest CasesArchive of Old Cases

Case No : 1237 Date 2021-07-05

  • Courtesy of Sung Hyun Yoon / Bundang Seoul National University Hospital
  • Age/Sex 37 / F
  • Chief ComplaintSPN in the RLL, serum hCG level elevation (about 20 mIU/ml). s/p Recent Op. for endometriosis. history of pregnancy and delivery (x2)
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Metastatic epithelioid trophoblastic tumor (ETT)
Radiologic Findings
Fig. 1. An irregular small nodule in the right lower lobe (RLL) of the lung.
Fig. 2. After 5 months, the small nodule in the RLL slightly increased in size. One year later after hormonal therapy, CT image shows decreased volume of the nodule. After discontinuation of hormonal therapy, the nodule slightly re-increased size.
Fig. 3. Positron emission tomography-CT scan showing no hypermetabolic lesion other than a nodule in the RLL.
Brief Review
Gestational trophoblastic neoplasms comprise a heterogeneous group of trophoblastic tumors including choriocarcinomas, epithelioid trophoblastic tumors (ETTs), and placental site trophoblastic tumors. ETTs are extremely rare. They originate from intermediate trophoblastic cells of the chorion laeve. The uterus is the primary site of ETTs (40%), followed by the cervix (31%). The lung is the most common extrauterine site, accounting for 19% of the cases. Very few ETTs show aggressive clinical behavior, and the sites of metastases include the lung, small bowel, fallopian tube, broad ligament, and endocervix. Although ETTs are most commonly associated with prior term deliveries (43%), they have also been associated with molar pregnancies (39%) and abortions (18%) occurring 2–300 months (mean 76 months) after the antecedent gestational event. One possible pathogenesis of this rare clinical phenomenon is the original transformation of trophoblastic cells that were passed to the lungs during the antecedent pregnancy. Another hypothesis is that pulmonary lesions may be the consequence of spontaneous resolution of an antecedent uterine ETT. Both intrauterine and extrauterine ETTs cause elevated human chorionic gonadotrophin (hCG) levels, although the hCG levels are < 2500 mIU/mL in 69% of the cases. Whereas choriocarcinomas are chemosensitive, ETTs are relatively chemoresistant. Therefore, surgical resection remains the primary treatment modality. Lewin et al. reported three cases of isolated pulmonary lesions with elevated hCG levels but with no evidence of uterine disease. All three patients underwent lung resection and hysterectomy, and the pathology report supported the diagnosis of isolated pulmonary ETT. Although only two patients received adjunctive chemotherapy, all three patients were alive without evidence of disease after the surgical resection of the tumor. With the accurate diagnosis of ETT, chemotherapy has largely been reserved for the treatment of metastatic, recurrent, or surgically unresectable disease.
References
1. Lewin SN, Aghajanian C, Moreira AL, Soslow RA. Extrauterine epithelioid trophoblastic tumors presenting as primary lung carcinomas: morphologic and immunohistochemical features to resolve a diagnostic dilemma. Am J Surg Pathol. 2009 Dec;33(12):1809-14.
2. Scott EM, Smith AL, Desouki MM, Olawaiye AB. Epithelioid trophoblastic tumor: a case report and review of the literature. Case Rep Obstet Gynecol. 2012;2012:862472.
3. Lei W, Zhang F, Zheng C, Zhao C, Tu S, Bao Y. Metastatic epithelioid trophoblastic tumor of the lung: A case report. Medicine (Baltimore). 2018 Apr;97(16):e0306.
4. Kavurmacı Ö, Akçam Tİ, Ergönül AG, Akgün Kavurmacı S, Turhan K. A rare tumor metastasis of bilateral lung: Epithelioid trophoblastic tumor. Turk Gogus Kalp Damar Cerrahisi Derg. 2018 Apr 30;26(2):316-319.
5. Li JW, Hu CC, Shi HY, Wu RJ. Extrauterine epithelioid trophoblastic tumors presenting as lung mass: A case report and literature review. Medicine (Baltimore). 2019 Feb;98(5):e14010.
Keywords

No. of Applicants : 101

▶ Correct Answer : 2/101,  2.0%
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - Govt. Kilpauk Medical College, Chennai , India VIGNESH K
▶ Correct Answer as Differential Diagnosis : 6/101,  5.9%
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - Chonnam National University Hospital , Korea (South) SEUNG WAN KANG
▶ Semi-Correct Answer : 48/101,  47.5%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - Korea University Guro Hospital , Korea (South) LEE SEOKYOUNG
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Showa General Hospital , Japan MASAFUMI KAIUME
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - , Korea (South) HYEYOUNG CHOI
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - , United Kingdom SAMSON KADE
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - Osaka Red Cross Hospital , Japan YUSUKE UTSUNOMIYA
  • - National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
  • - Seoul National University Bundang Hospital , Korea (South) SOWON JANG
  • - , Italy PAOLO BALDASSARI
  • - , Japan CHIAKI SATO
  • - Ichinomiya Nishi Hospital , Japan CHIHIRO KIGUCHI
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - , Korea (South) JIN YOUNG LEE
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - Gifu University Hospital , Japan Yo Kaneko
  • - VDC, HYDERABAD , India KARTHIK RAYASAM
  • - , Japan KANAE TAKAHASHI
  • - , Korea (South) EUNJIN LEE
  • - Mallinckrodt Institute of Radiology , United States NAGANATHAN MANI
  • - Other , Korea (South) MINSU KIM
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - AIMS , India NITHYA HARIDAS MALIYAM
  • - CHU Lille , France PAUL LEBERT
  • - , Korea (South) YEJIN JEON
  • - Osmania Medical College, Hyderabad , India PURUSHOTHAMA RAO TUMMALA
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - Kyoto University Hospital , Japan SATOSHI IKEDA
  • - Samsung Medical Center , Korea (South) SUHO KIM
  • - Pusan National University Yangsan Hospital , Korea (South) JI HWAN KIM
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - , Japan KAZUMA TERAUCHI
  • - National Center for Global Health and Medicine , Japan TOMOKI IMOKAWA
  • - Chonbuk National University Hospital , Korea (South) YOUNG JU SONG
  • - Private sector , Greece VASILIOS TZILAS
  • - , Japan YUKI HAYASHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Kyoto university , Japan AKIHIKO SAKATA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - algeria , Algeria ZAKARIA CHERAGA
  • - Jiangsu province hospital , China WANGJIAN ZHA
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