Parenchymatous pulmonary endometriosis – metastases of a low-grade endometrial stromal sarcoma?

Parenchymatous pulmonary endometriosis – metastases of a low-grade endometrial stromal sarcoma?

Medical Hypotheses (2003) 61(5–6), 651–653 ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/S0306-9877(03)00268-8 Parenchymatous pulmonary endom...

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Medical Hypotheses (2003) 61(5–6), 651–653 ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/S0306-9877(03)00268-8

Parenchymatous pulmonary endometriosis – metastases of a low-grade endometrial stromal sarcoma? Klaus Bodner, Martha Zauner, Barbara Bodner-Adler, Babette Spa¨ngler, Werner Gru¨nberger, Franz Wierrani Department of Gynecology and Obstetrics, Hospital Rudolfstiftung, Vienna, Austria

Summary Based on the data of a case of parenchymatous endometriosis of the lung in which three and a half years later a low-grade endometrial stromal sarcoma of the uterus was diagnosed the origin of this disease is discussed. The follow up of this patient gave the impression that the pulmonary nodules were early metastases of low-grade endometrial stromal sarcoma, which was initially not detected in the routine gynecologic examination. Furthermore, as histological and immunhistochemical examinations are not sufficient to distinguish reliable between endometriosis of the lung and pulmonary metastases from low-grade endometrial stromal sarcoma. In literature, in none of the few cases of parenchymatous pulmonary endometriosis a hysterectomy was performed to exclude a low-grade endometrial stromal sarcoma. Thus, parenchymatous pulmonary endometriosis nodules might be metastases and their occurrence should cause the treating physician to consider a distant metastatic spread from low-grade endometrial stromal sarcoma. ª 2003 Elsevier Ltd. All rights reserved.

INTRODUCTION Pleural endometriosis is accompanied by pelvic endometriosis in over 90% of the recorded cases (1). Usually, this type is located in the right hemithorax and is detected because symptoms like pneumothorax or haemoptysis occur on the average 5 years post initial diagnosis of pelvic endometriosis (2). In literature two theories about the origin of pleural endometriosis have been published: the metastatic theory of endometrioid

Received 12 December 2002 Accepted 30 July 2003 Correspondence to: Klaus Bodner MD, Department of Gynecology and Obstetrics, Hospital Rudolfstiftung, A-1030 Vienna, Juchgasse 25, Austria. Phone: +43 1 71165 4708; Fax: +43 1 71165 4709; E-mail: [email protected]

tissue through the diaphragm and the metaplastic theory of pluripotent coelomaepithelial cells (3). Parenchymatous endometriosis occurs in single lesions; in this case an additional pelvic endometriosis can rarely be detected (1–4). In case of parenchymatous endometriosis a much too forceful filter function of the vascular network of the lung has been suggested (5). After excessive research of the literature the authors found only one study dealing with the topic of nodular, parenchymatous endometriosis with follow-up data of up to 5 years (4). Worldwide less than 25 reports of histological verified cases are described in literature (4–21). A malignant degeneration of endometriosis seems to be a very rare event. Less than 50 cases of malignant, non-ovarian endometriosis genitalis externa have been published in literature (22,23). Additionally in 70 proven cases endometriosis prepared the ground for ovarian

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cancer (24). A sarcomatous degeneration of endometriosis is supposed to be an extremely rare event that happens due to malignant degeneration of cytogene stroma components (25). However, the combined appearance of endometriosis and epithelial or non-epithelial neoplasm should not immediately lead to the conclusion of malignant metaplasia of endometriosis. A coincidence of ovarian endometriosis and endometrioid ovarian cancer occurs for example in 11–28% (26). Stromatogene mesenchymal uterine tumors include endometrial stromal sarcomas, which are divided into low- and high-grade endometrial stromal sarcomas depending on their mitotic count. This rare subset of uterine sarcomas demonstrates persistent stromatogene differentiation with high mitotic activity. Low-grade endometrial stromal sarcomas tend to grow slowly with late metastatic spread (27). Pulmonary metastases mainly occur as a late recurrence disease due to haematogenous spread (27). Histologically, the diagnosis and classification of these pure mesenchymal tumors is reliable. However, clinically without excessive pathologic examination of the slides the diagnostic instruments are not sufficient to make a reliable differentiation between endometriosis of the lung and pulmonary metastases from low-grade endometrial stromal sarcomas. CASE REPORT AND HYPOTHESIS Our oncologic research team published two case reports about a patient with multiple endometrial stromal nodules with sparse cysts and glands in the lung – a nodular variation of endometriosis that may mimic metastasis of sarcoma (3,28). The patient presented at the gynecologic outpatient department of the Hospital Rudolfstiftung with nodular parenchymatous endometriosis of the lung that was detected purely accidental by chest X-ray before a planned appendectomy (28). The diagnosis was verified by open thoracoscopy. Because of the histopathologic structure of the tumor cells, the strong positive immunhistochemical staining reaction of the stromal cells against ERICA and the negative reaction against CD 34 fibrous tumors of the pleura could be excluded (3,29). Cysts and glands were lined with a monolayer of flat, cuboidal and columnar cells. Immunhistochemically, these cells demonstrated a strong positive staining reaction with BerEp 4, Cytoceratin AE1/AE3 and Cytoceratin PAN (3). Cytophotometry of these cells revealed a diploid chromosome arrangement (3,30). Due to inconspicuous gynecologic examination (with deep, intracervical exfoliativcytology and vaginal sonography) we first did not think about a metastatic process resulting from a low-grade endometrial stromal Medical Hypotheses (2003) 61(5–6), 651–653

sarcoma. With diagnosis nodular parenchymatous endometriosis of the lung the patient was set on a successful therapy regimen consisting of GnRH-analogue (gosereline acetate) and high doses of gestagen (28). Three and a half years past initial thoracoscopy a uterine nodule was detected and a laparascopic enucleation of the suggested uterine leiomyoma was performed. Histologically and immunhistochemically the tumor was classified as a low-grade endometrial stromal sarcoma so that an abdominal hysterectomy and bilateral adnexectomy had to be performed. The gross examination of the uterus revealed additional stroma nodules. Immunhistochemically, the tumor lesions, which were infiltrated with lymphocytes, revealed a strong positive staining reaction for vimentin and a weak positive for estrogen and progesterone. The myometrium showed a strong positive staining reaction for Desmin and SMA, whereas the parts of the tumor did not stain. The coincidence of parenchymatous endometriosis of the lung and low-grade endometrial stromal sarcoma in our patient means that two extremely rare diseases were diagnosed within three years (3,28). The follow up of this case gave us the impression that low-grade endometrial stromal sarcoma, which was initially not detected in the routine gynecologic examination, showed an early metastatic spread to the lung. Furthermore, as histological and immunhistochemical examinations are not sufficient to distinguish reliable between endometriosis of the lung and pulmonary metastases from lowgrade endometrial stromal sarcomas. Another argument for this hypothesis could be that pulmonary metastases of low-grade endometrial stromal sarcoma as well as pulmonary endometriosis respond to high doses of gestagen (31,32) and GnRH-analogue (33,34). Our patient was set on a therapy consisting of GnRH-analogue (gosereline acetate) and high doses of gestagen that led to a reduction of the tumor nodules in the lung. This therapeutic regimen was probably also the reason for the slow growth of the low-grade endometrial stromal sarcoma and the late detection of this disease (312 years after initial thoracoscopy). CONCLUSION We believe that the few cases of parenchymatous endometriosis reported in literature could be early metastases of a non-recognized low-grade endometrial stromal sarcoma (35). In none of the cases of parenchymatous endometriosis reported in literature a hysterectomy was performed to exclude a low-grade endometrial stromal sarcoma. Thus, the occurrence of nodular lung endometriosis should cause the treating physician to consider a distant metastatic spread from low-grade endometrial stromal sarcoma. ª 2003 Elsevier Ltd. All rights reserved.

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REFERENCES 1. Gaetje R., Kotzian S., Herrman G., Baumann R., Starzinski Powitz A. Invasiveness of endometroid cells in vitro. Lancet 1995; 364: 1463–1464. 2. Joseph J., Sahn S. A. Thoracic endometriosis syndrome: New observations from an analysis of 110 cases. Am J Med 1996; 100: 164–170. € hm G., Hauck H., Lintner F., 3. Huber M., Wierrani F., Bo € nberger W. Multiple endometrial stromal nodules with Gru sparce cysts and glands in the lung – a nodular variation of endometriosis that may mimic metastases of sarcoma. Pathol Res Pract 1999; 195: 59–64. 4. Flieder D. B., Moran C. A., Travis W. D., Koss M. N., Mark E. J. Pleuro-pulmonary endometriosis and pulmonary ectopic deciduosis: A clinicopathologic and immunohistochemical study of 10 cases with emphasis on diagnostic pitfalls. Hum Pathol 1998; 29: 1495–1503. 5. Park W. The occurence of decidual tissue within the lung: An experimental and clinical study. Am J Obstet Gynecol 1940; 40: 832–843. 6. Lattes R., Shepard F., Tovel H. et al. A clinical and pathologic study of endometriosis of the lung. Surg Gynecol Obstet 1956; 103: 552–558. 7. Austin M. B., Frierson H. F., Jr, Frechner R. E. et al. Endometrioma of the lung presenting as hemoptysis and a large pulmonary mass. Surg Pathol 1988; 1: 165–169. 8. Kristiansen K., Fjeld N. B. Pulmonary endometriosis causing haemoptysis: Report of a case treated with lobectomy. Scand J Thorac Cardiovasc Surg 1993; 27: 113–115. 9. Cassina P. C., Hauser M., Kacl G. et al. Catamenial hemoptysis: Diagnosis with MRI. Chest 1997; 111: 1447–1450. 10. Rodman M. H., Jones C. W. Catamenial hemoptysis due to bronchial endometriosis. N Engl J Med 1962; 266: 805– 808. €ssler R., Ku € mmerle D. et al. Endometriose der 11. Egidy H. V., Ba € hnlicher Lunge. Ein Beitrag zur Differentialdiagnose ungewo €moptysen. Dtsch Med Wochenschr 1967; 92: 1220– Ha 1225. 12. Assor D. Endometriosis of the lung: Report of a case. Am J Clin Pathol 1972; 57: 311–315. 13. Lindenberg K., Schmid J., Ruttner J. et al. Endometriosis of the lung: Case Report. Arch Gynecol 1975; 218: 219–226. 14. Sugimani H., Hamada K., Yano K. A case of endometriosis of the lung treated with danazol. Obstet Gynecol 1985; 66: 688–718. 15. Sturzenegger H. Lungenendometriose unter dem Bild des Rundschattens. Schweiz Z Tuberk 1960; 17: 259–266. 16. Mobbs G. A., Pfanner D. W. Endometriosis of the lung. Lancet 1963; 1: 472–474. 17. Jelihovshy T., Grant A. F. Endometriosis of the lung: A case report and brief review of the literature. Thorax 1968; 23: 434–437. 18. Granberg I., Willems J. S. Endometriosis of the lung and pleura diagnosed by aspiration biopsy. Acta Cytol 1977; 21: 295–297. 19. Duskova J., Vitkova I. Pulmonary endometriosis. Cas Lek Cesk 2001; 140: 214–216.

ª 2003 Elsevier Ltd. All rights reserved.

20. Saint-Blancard P., Simon F., Jancovici R., Vaylet F., L’her P., Le Vagueresse R. Unusual pulmonary nodules. Ann Pathol 2001; 21: 271–272. 21. Weber F. Catamenial hemoptysis. Ann Thorac Surg 2001; 72: 1750–1751. 22. Grimes D. A., Fowler W. C., Jr Adenosquamous carcinoma of the cecum arising in endometriosis. Gynecol Oncol 1980; 9: 254–255. 23. Shamsuddin A. K., Villa Santa U., Tang C. K., Mohamed N. C. Adenocarcinoma arising from extragonadal endometriosis 14 years after totalhysterectomy and bilateral salpingo-oophorectomy for endometriosis: Report of acase with ultrastructural studies. Am J Obstet Gynecol 1979; 133: 585–586. 24. Kobayashi F., Monma C., Nanbu K. et al. Rapid growth of an ovarian clear cell carcinoma expressing LH/hCG receptor arising from endometriosis during early pregnancy. Gynecol Oncol 1996; 62: 309–313. 25. Crum C. P., Wible J., Frick H. C., Fenoglio C. M., Richart R. M., Williamson S. A case of extensive pelvic endometriosis terminating in endometrial sarcoma. Am J Obstet Gynecol 1981; 140: 718–719. 26. Mostoufizadeh M. G. H., Scully R. E. Malignant tumors arising from endometriosis. Clin Obstet Gynecol 1980; 23: 951–963. 27. Bodner K., Bodner-Adler B., Obermair A., Windbichler G., Petru E., Mayerhofer S. et al. Prognostic parameters in endometrial stromal sarcoma: A clinicopathologic study in 31 patients. Gynecol Oncol 2001; 81: 160–165. €ngler B., 28. Fousek C., Huber M., Hinterberger H., Spa € nberger W., Wierrani F. Die Therapie einer inoperablen, Gru €ren Lungenendometriose mit Goserelin. Geburtsh nodula Frauenheilk 2000; 60: 229–231. 29. Renshaw A. A., Pinkus G. S., Corson M. C. CD34 and AE1/ AE3. Diagnostic discriminants in the distinction of solitary fibrous tumor of the pleura from sarcomatoid mesothelioma. Appl Immunhistochem 1994; 2: 94–102. 30. Goldfarb S., Richart R. M., Okagaki T. Nuclear DNA content in endolyphatic stromal myosis. Am J Obstet Gynecol 1970; 106: 524–529. 31. Pellilo D. Proliferative stromatosis of the uterus with pulmonary metastasis. Remission following treatment with a long acting synthetic progestins: A case report. Obstet Gynecol 1968; 31: 33–39. 32. Krumholz B. A., Lobovsky F. Y., Halitsky V. Endolymphatic stromal myosis with pulmonary metastases. Remission with progestintherapy: Report of a case. J Reprod Med 1973; 10: 85–89. 33. Mesia A. F. Demopoulos RI. Effects of leuprolide acetate on low-grade endometrial stromal sarcoma. Am J Obstet Gynecol 2000; 182: 1140–1141. 34. Koizumi T., Inagaki H., Takabayashi Y., Kubo K. Successful use of gonadotropin-releasing hormone agonist in a patient with pulmonary endometriosis. Respiration 1999; 66: 544–546. 35. Levine P. H., Abou-Nassar S., Mittal K. Extrauterine lowgrade endometrial stromal sarcoma with florid endometrioid glandular differentiation. Int J Gynecol Pathol 2001; 20: 395–398.

Medical Hypotheses (2003) 61(5–6), 651–653