This article has Open Peer Review reports available.
Safe and successful birth following pelvic radiotherapy for rectal mucosa-associated lymphoid tissue lymphoma: a case report
© The Author(s). 2017
Received: 24 October 2016
Accepted: 27 December 2016
Published: 1 February 2017
Mucosa-associated lymphoid tissue lymphomas can occur in various parts of the body, and half of mucosa-associated lymphoid tissue lymphomas occur in the gastrointestinal tract. Gastric mucosa-associated lymphoid tissue lymphoma is the most common lymphoma of the gastrointestinal tract and primary rectal mucosa-associated lymphoid tissue lymphoma is very rare. Because of the high radiosensitivity of mucosa-associated lymphoid tissue lymphomas, this condition can be controlled with radiotherapy of approximately 30 Gy alone. However, ovarian dysfunction as an adverse event of radiotherapy for pelvic lesions can become a problem in girls and women. We report a case of a 28-year-old woman with rectal mucosa-associated lymphoid tissue lymphoma who safely gave birth to a baby following 30.6 Gy radiotherapy to her whole rectum.
A 28-year-old Japanese woman became aware of bloody stools and was diagnosed as having Lugano I rectal mucosa-associated lymphoid tissue lymphoma. She was referred to our institute and initiated on radiotherapy. However, she expressed a desire to bear children. We used horizontally opposed pair fields for radiotherapy to minimize the irradiation to her endometrium and ovary. A total dose of 30.6 Gy was given in 17 fractions of 1.8 Gy by 10-Megavolt X-ray linear accelerator. As a result, one-third of her uterus and half of her ovary were outside the irradiation field. After approximately 1 year of treatment, positive pregnancy was confirmed and finally she safely gave birth to a baby girl without congenital abnormalities.
This report provides hope for girls and women who have undergone irradiation for pelvic mucosa-associated lymphoid tissue lymphomas and who desire to bear children.
Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade B cell lymphoma, first identified by Isaacson and Wright in 1983 . This lymphoma can occur in various parts of the body, and half of all MALT lymphomas occur in the gastrointestinal tract. Gastric MALT lymphoma is the most common gastrointestinal tract lymphoma, comprising 85% of all cases, and primary rectal MALT lymphoma is very rare [2, 3]. Because of the low frequency of the disease, the optimal therapeutic strategy for primary non-gastric lymphomas is yet to be established; however, prognosis is good, and the 5-year and 10-year overall survival rates have been reported to be approximately 90% and 80%, respectively [4, 5]. Radiotherapy or surgical resection is considered to be a therapeutic strategy of limited stage MALT lymphomas, but because of the high radiosensitivity of MALT lymphomas, this condition can be controlled by radiotherapy of approximately 30 Gy alone [6–8]. The present case was of a 28-year-old Japanese woman who desired to bear children. However, ovarian dysfunction as an adverse event of radiotherapy for pelvic lesions can become a serious problem. Here we report a case of a 28-year-old woman with rectal MALT lymphoma who safely gave birth to a baby following 30.6 Gy radiotherapy to her whole rectum.
She was then introduced to our institute and initiated on radiotherapy. However, she expressed a desire to bear children and had some embryos frozen by the Department of Obstetrics and Gynecology before being admitted to our department in February 2014.
She completed radiotherapy and was discharged in March 2014, but returned to the Department of Obstetrics and Gynecology in May 2014 due to amenorrhea. A female hormone test revealed a reduction in estrogen level and an increase in follicle-stimulating hormone level. She was thus diagnosed as having premature ovarian failure. Kaufmann therapy was then commenced. After approximately 1 year of treatment, positive pregnancy was confirmed (April 2015), and she revisited the clinic. A gestational sac was confirmed by ultrasound examination, and the second month of pregnancy was diagnosed. Surprisingly, she had conceived naturally. Finally, she safely gave birth to a baby girl without congenital abnormalities in October 2015.
Non-gastric MALT lymphomas can occur in the colon, lung, thyroid gland, salivary glands, mammary gland, and ophthalmologic fields. Primary rectal MALT lymphoma is a particularly rare disease, accounting for less than 1% of all MALT lymphomas [2, 3]. Rectal MALT lymphomas tend to show elevated lesions, such as polyps or submucosal tumors, during endoscopy, and our case presented with similar characteristic polypoid lesions . Because of the low incidence of the disease, conducting large clinical trials is difficult. Therefore, the optimal therapeutic strategy for this type of lymphoma is yet to be established, and therapeutic strategy is currently determined according to the lesion site or clinical stage. In the case of limited stage MALT lymphomas, local therapy, such as radiotherapy or surgery, is generally the principal course of action. However, MALT lymphomas can be well controlled by radiotherapy alone with approximately 30 Gy, and surgery is considered excessive with regard to organ preservation and the postoperative quality of life. The therapeutic strategy used in the present case was radiation therapy, and a total dose of 30.6 Gy was given in 17 fractions to the whole rectum. After 9 months of radiotherapy, our patient was endoscopically examined, and all the lesions had undergone complete remission. No severe gastrointestinal late adverse events, such as rectal bleeding, have yet been observed.
A critical point of this case was our patient’s desire to bear children, and we wanted to meet her desire, as well as gain good control over her rectal MALT lymphoma. However, when radiotherapy is focused onto the pelvic cavity, ovarian dysfunction as an adverse event of radiotherapy becomes a serious problem. Therefore, we performed radiotherapy using horizontally opposed pair fields, which goes against the era of three-dimensional radiotherapy. Our treatment procedure had two purposes. First, we tried to minimize the amount of irradiation exposure to her ovaries. Although she had frozen embryos stored, we wanted to preserve her ovarian function as much as possible in an effort to give her a chance of a natural pregnancy. Second, we attempted to minimize the amount of irradiation exposure to her endometrium, such that we could maximize the chance of normal implantation if she could not become pregnant naturally and needed to use her frozen embryos.
Schuck et al. reported that when the whole ovary is irradiated more than 15 Gy, ovarian dysfunction occurs in almost all patients. In addition, when one side of the ovary is preserved, ovarian dysfunction occurs in approximately half of all patients . The mean dose absorbed by the ovary in this patient’s case was 16.6 Gy, although our treatment procedure was successfully achieved by removing half of her ovary from the irradiation field. Therefore, half of her ovary was not completely irradiated, and we expected her ovarian function to be preserved with a probability of 50%. Following our procedure, she developed temporary ovarian dysfunction; Kaufmann therapy, a replacement therapy comprising estrogen and progesterone for ovarian amenorrhea, was commenced. We consider that preserving half of her ovary using horizontally opposed pair fields for radiotherapy was a key benefit in terms of ovulation and natural pregnancy. On the other hand, the tolerance dose of the endometrium is not yet known and, consequently, there is a potential risk of implantation disorders. Because we could not identify her endometrium by CT, the irradiation dose to her endometrium could not be determined using a dose volume histogram. However, we believe that removing one-third of her uterus from the irradiation field was a key contributor to retaining normal implantation, thereby leading to the safe birth of a baby girl. Although the full dose of 30.6 Gy was used to irradiate her vagina, she delivered her baby without any problem. Therefore, we consider a total dose of approximately 30 Gy does not cause adverse effects to the vagina.
Here we report the case of a 28-year-old woman with a rectal MALT lymphoma who safely gave birth to a baby following 30.6 Gy radiotherapy to her whole rectum. This case involves several rare points. First, there is a low incidence of primary rectal MALT lymphoma among cases of MALT lymphoma. Second, ovarian function could be preserved by removing half of her ovary from the irradiation field using horizontally opposed pair fields. Finally, by removing one-third of her uterus from the irradiation field, we could preserve normal implantation, which enabled her to give birth naturally. We believe that this case is extremely rare and will be of global interest, and we are proud to be able to report the control of rectal MALT lymphoma and the safe birth of a healthy child in this 28-year-old woman.
The authors have no sources of funding to declare.
Availability of data and materials
All authors reviewed and contributed to the final version of this case report. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Ethics approval and consent to participate
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
- Isaacson P, Wright DH. Malignant lymphoma of mucosa-associated lymphoid tissue. A distinctive type of B-cell lymphoma. Cancer. 1983;52:1410–6.View ArticlePubMedGoogle Scholar
- Takenaka R, Tomoda J, Sakata T, et al. Mucosa-associated lymphoid tissue lymphoma of the rectum that regressed spontaneously. J Gastroenterol Hepatol. 2000;15:331–5.View ArticlePubMedGoogle Scholar
- Tsang RW, Gospodarowicz MK, Pintilie M, et al. Stage I and II MALT lymphoma: results of treatment with radiotherapy. Int J Radiat Oncol Biol Phys. 2001;50:1258–64.View ArticlePubMedGoogle Scholar
- Thieblemont C. Clinical presentation and management of marginal zone lymphomas. Hematology Am Soc Hematol Educ Program. 2005:307–13.Google Scholar
- Zucca E, Conconi A, Pedrinis E, et al. Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood. 2003;101(7):2489–95.View ArticlePubMedGoogle Scholar
- Goda JS, Gospodarowicz MK, Pintilie M, et al. Long-term outcome in localized extranodal mucosa-associated lymphoid tissue lymphomas treated with radiotherapy. Cancer. 2010;116(16):3815–24.View ArticlePubMedGoogle Scholar
- Isobe K, Kagami Y, Higuchi K, et al. A multicenter phase II study of local radiation therapy for stage IEA mucosa-associated lymphoid tissue lymphomas: a preliminary report from the Japan Radiation Oncology Group (JAROG). Int J Radiat Oncol Biol Phys. 2007;69(4):1181–6.View ArticlePubMedGoogle Scholar
- Schechter NR, Portlock CS, Yahalom J. Treatment of mucosa-associated lymphoid tissue lymphoma of the stomach with radiation alone. J Clin Oncol. 1998;16(5):1916–21. doi:10.1200/jco.19188.8.131.526. PMID: 9586910, https://www.ncbi.nlm.nih.gov/pubmed/9586910.PubMedGoogle Scholar
- Kobayashi T, Takahashi N, Hagiwara Y, et al. Successful radiotherapy in a patient with primary rectal mucosa-associated lymphoid tissue lymphoma without the AP12-MALT1 fusion gene: A case report and review of the literature. Leuk Res. 2008;32(1):173–5.View ArticlePubMedGoogle Scholar
- Schuck A, Hamelmann V, Brämswing JH, et al. Ovarian function following pelvic irradiation in prepubertal and pubertal girls and young adult women. Strahelenther Onkol. 2005;181(8):534–9.View ArticleGoogle Scholar