Japanese Journal of Clinical Oncology 32:71-74 (2002)
© 2002 Foundation for Promotion of Cancer Research
Spontaneous Regression of Metastatic Endometrial Stromal Sarcoma
1Second Department of Internal Medicine and 4Second Department of Pathology, Okayama University Medical School, Okayama, 2Department of Internal Medicine, Kagawa Rousai Hospital, Kagawa, 3Department of Pathology, Okayama University Hospital, Okayama, 5Department of Internal Medicine, Okayama Rousai Hospital, Okayama and 6Department of Internal Medicine, Tsuyama Central Hospital, Tsuyama, Japan
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ABSTRACT |
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 TOP ABSTRACT INTRODUCTIONCASE REPORTDISCUSSIONAcknowledgmentsREFERENCES |
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Spontaneous regression of malignancy is rare and there appear to be no reports of spontaneous regression of endometrial stromal sarcoma. We report a rare case of metastatic endometrial stromal sarcoma that regressed spontaneously. A 58-year-old woman was admitted to hospital in January 1996 when her chest radiograph showed multiple nodular shadows in the left lower lung field. Computed tomography of the chest revealed bilateral nodules. Segmentectomy of the left lower lobe was performed by thoracoscopy. She had a past history of uterine myoma with metrorrhagia for which she had undergone a hystero-oophorectomy 10 years earlier. She also had a vaginal polyp removed 1 year earlier. The lung pathology was studied and the surgical specimens of the uterus and vagina were re-examined. The diagnosis was endometrial stromal sarcoma primarily arising in the uterus. The vaginal polyp and the pulmonary nodules were considered to be metastases. Samples of lung and vaginal tissues were positive for both estrogen and progesterone receptors. The patient was discharged without treatment in February 1996 and followed up in the outpatient clinic. The tumor shadow measuring 2 mm in diameter on admission was enlarged to 4 mm in diameter 1 year later. Surprisingly, spontaneous regression of the lung disease occurred at 33 months, the tumor size decreasing to 2 mm in diameter and to 1 mm at 46 months. No evidence of tumor enlargement was detected at the last follow-up in July 2001. Although the precise mechanism of tumor regression is unknown, metastatic endometrial stromal sarcoma may spontaneously regress.
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INTRODUCTION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONAcknowledgmentsREFERENCES |
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Spontaneous regression of cancer is defined as ‘the partial or complete disappearance of a malignant tumor in the absence of all treatment or in the presence of therapy which is considered inadequate to exert a significant influence on neoplastic disease’ (1). The incidence of spontaneous regression has been estimated to be not more than 1 in 60 000–100 000 (2).
Endometrial stromal sarcoma (ESS) is a uterine sarcoma which has never been reported to regress spontaneously. We report here a case of metastatic ESS that regressed spontaneously.
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CASE REPORT |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONAcknowledgmentsREFERENCES |
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A 58-year-old woman was admitted to a local hospital in October 1995 because abnormal shadows had been detected in the left lower lung field on a chest radiograph at her annual check-up. Computed tomographic (CT) scans of the chest disclosed multiple nodular shadows scattered in the bilateral lung fields (Fig. 1a). Resection of a nodule in the left lower lobe was performed by thoracoscopic surgery in December (Fig. 2). The histology of the lung surgical specimen was consistent with metastatic tumor. She was referred to us 1 month later for consultation. The patient had experienced metrorrhagia and undergone hystero-oophorectomy in 1987. Histopathological diagnosis was uterine myoma. She had also had a vaginal polypectomy 1 year earlier. The histopathological diagnosis was leiomyoblastoma.
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On admission she was asymptomatic. Physical examination revealed no significant findings. Hematological and blood chemistry tests were within normal limits. Serum concentrations of conventional tumor markers were not elevated. The surgical specimens of the lung (Fig. 2), uterus (Fig. 3a) and vagina (Fig. 4) that had been obtained previously were re-examined. All of these specimens showed similar histological findings, namely small cells, which were uniform in size and shape with bland nuclei, whirling around small blood vessels. Mitotic figures were found in 1/10 hpf at most in the lung, 4/10 hpf at most in the uterus and 6/10 hpf at most in the vagina. The histological diagnosis was low-grade endometrial stromal sarcoma arising in the uterus. The vaginal polyp and pulmonary nodules were considered metastatic lesions of the ESS. Samples of lung and vaginal tissues (Fig. 5) were positive for both estrogen and progesterone receptors.
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The patient was discharged in February 1996, not having received any treatment including hormonal therapy against ESS, and was followed monthly in our outpatient clinic. One year later, tumor shadows showed enlargement on chest CT scans (Fig. 1b), but they regressed spontaneously without any treatment over 18 months (Fig. 1c). Tumor enlargement was not detected by CT scans in December 1999 (Fig. 1d) and at the last follow-up in July 2001. The white arrow in Fig. 1 indicates a tumor shadow measuring ~2 mm in diameter in (a), enlarged to 4 mm in (b), regressed to 2 mm in (c) and to 1 mm in (d). The black arrow in Fig. 1 indicates a tumor shadow measuring ~2 mm in diameter in (a), continuing at 2 mm in (b), regressed to 1 mm in (c) and disappeared in (d). Other lesions regressed at a rate similar to that identified by the white and black arrows.
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DISCUSSION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONAcknowledgmentsREFERENCES |
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Endometrial stromal sarcomas are usually designated as low grade (formerly termed endolymphatic stromal myosis) or high grade. They tend to present in the fifth decade. Low-grade stromal sarcomas usually grow slowly and tend to recur years after the initial treatment, usually hysterectomy, in some cases up to 25 years later. Most recurrences are in the pelvis, but pulmonary metastases develop in up to 45% of the patients (3).
The present case is important because of the spontaneous regression of metastasic lesions. This patient was discharged without any treatment, because chemotherapy and hormonal therapy are reported to be not very effective against this disease (4). Interestingly, the patient maintained a durable regression. We searched Medline back to 1966 and found no reports of spontaneous regression of ESS.
A variety of mechanisms, including immunological and hormonal factors, have been proposed as a mechanism for the spontaneous regression of cancer. Hormonal manipulation causes regression in almost 50% of prostatic cancers, 30–40% of breast cancers and 30% of endometrial cancers (5).
Cole reviewed 176 cases of spontaneous regression of cancer (2). Almost half of the cases involved cancer of the kidney, neuroblastoma or melanoma. Seventy-one patients had undergone some type of operation and eight had experienced severe infection before spontaneous regression. In five cases, spontaneous regression was thought to be caused by hormonal effects. Cole suggested that stimulation of the immune system is the most important factor in spontaneous regression of cancer. However, the patient described here experienced no particular event which might have affected her immune system.
Reich et al. analyzed estrogen receptor (ER) and progesterone receptor (PR) expression in a retrospective series of 21 low-grade ESS. ER were seen in 15 (71%) and PR in 20 (95%) of tumors (6). In our case, samples of lung and vaginal tissues (Fig. 5) were positive for both estrogen and progesterone receptors. Estrogen and progesterone are important regulators of endometrial stromal function and act by binding to their nuclear receptors. However, the patient had not received hormonal therapy against ESS.
Mackensen et al. derived a series of T-cell receptor 
/ß-dependent, HLA-B14-restricted cytotoxic T-lymphocyte clone reactive against an autologous tumor from lymphocytes which were infiltrating a regressing melanoma lesion (7). Their study provides evidence that effector T-cells may contribute to tumor regression. We speculate that a similar event might have occurred in our case.
In conclusion, although the precise mechanism of tumor regression is uncertain, our case indicates that ESS may spontaneously regress.
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Acknowledgments |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONAcknowledgmentsREFERENCES |
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We thank Mrs McCown and Dr Zensuke Ota for their invaluable advice. We are also grateful to Ms Takabatake, Ms Saito, Ms Mizuuchi, Mr Okamoto and Ms Tomosada for their excellent assistance.
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FOOTNOTES |
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+ For reprints and all correspondence: Seisuke Ota, Himeji St. Mary’s Hospital, 650 Nibuno, Himeji, Hyogo 670-0801, Japan. E-mail: seisukeota@mail.goo.ne.jp
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REFERENCES |
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1 Everson TC, Cole WH. Anonymous Spontaneous Regression of Cancer. Philadelphia: Saunders 1966:1–10, 164–220.
2 Cole WH. Efforts to explain spontaneous regression of cancer. J Surg Oncol 1981;17:201–9.[Web of Science][Medline]
3 Byers LJ, Fowler JM, Twiggs LB. Uterus. In: Abeloff MD, Armitage JO, Lichter AS, Niederhuber JE, editors. Clinical Oncology. New York: Churchill Livingstone 1995:1577–8.
4 Sutton G, Blessing JA, Park R, DiSaia PJ, Rosenshein N. Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol 1996;87:747–50.[Web of Science][Medline]
5 Stoll BA. Spontaneous regression of cancer: new insights. Biotherapy 1992;4:23–30.[Web of Science][Medline]
6 Reich O, Regauer S, Urdl W, Lahousen M, Winter R. Expression of oestrogen and progesterone receptors in low-grade endometrial stromal sarcomas. Br J Cancer 2000;82:1030–4.[Web of Science][Medline]
7
Mackensen A, Ferradini L, Carcelain G, Triebel F, Faure F, Viel S, et al. Evidence for in situ amplification of cytotoxic T-lymphocytes with antitumor activity in a human regressive melanoma. Cancer Res 1993;53: 3569–73.
Received April 16, 2001; accepted November 19, 2001.
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