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Pure infra-diaphragmatic presentation is rare in pediatric Hodgkin lymphoma (HL). Isolated pelvic presentation is restricted to case reports in adults. We describe a unique presentation of HL as a primary pelvic disease in an 11-year-old prepubertal girl. She was treated based on the Euronet-Paediatric Hodgkin's Lymphoma Group protocol (EuroNet-PHL-C1) recommendations and assigned to treatment Group 2 (Stage IIB). The patient had bulky disease and a suboptimal response on interim 18-fluorodeoxyglucose positron emission tomography. The child was administered chemotherapy alone. Pelvic radiotherapy was consciously avoided due to the high risk of gonadal failure. She remains disease-free for 18 months now. Treatment of pediatric HL necessitates a delicate balance between achieving cure as well as avoiding serious late effects of therapy.
Primary sub-diaphragmatic pediatric Hodgkin lymphoma (HL) comprises merely 3% of all pediatric cases. Isolated HL in the pelvis is rarely reported.[2,3] We report a prepubertal girl with a pelvic mass, which uniquely turned out to be HL. Besides chemotherapy, the management involved debating pros and cons of administering radiotherapy to the sensitive site.
An 11-year-old girl presented with fever for 4 months. On examination, a left-sided suprapubic mass was palpated. Ultrasonography confirmed a mass in the pelvis. A magnetic resonance imaging revealed a large mass located in the pelvis, along with enlarged lymph nodes [Figure 1]. The initial clinical possibility was of a gonadal germ cell tumor. Serum alpha-fetoprotein and beta-human chorionic gonadotrophin were normal. A core biopsy was consistent with a diagnosis of HL, nodular sclerosis (NS) type. Immunohistochemistry was confirmatory; the large atypical cells were positive for CD15/CD30 and negative for CD20. An 18-fluorodeoxyglucose-positron emission tomography-computed tomography (FDG-PET-CT) confirmed FDG avidity of the mass and adjoining nodes. The bone marrow was not infiltrated.
The patient was assigned to stage IIB and treatment - Group-2 of the Euronet-Paediatric Hodgkin's Lymphoma Group protocol (EuroNet-PHL-C1). Two cycles of O-vincristine, E-etoposide, P-prednisone, A-adriamycin were administered. An interim FDG-PET-CT revealed >50% reduction of the mass, with a Deauville score (DS) of three, with complete resolution of the nodes. As per the guidelines of EuroNet-PHL-C1 trial, with a local partial remission and a DS of three, radiotherapy was indicated following two cycles of C-cyclophosphamide, O-vincristine, P-prednisone, DAC-dacarbazine (COPDAC). However, there was a major concern for infertility with radiotherapy to the pelvis. The multi-disciplinary team, along with the parents debated the options. The emerging international consensus was of DS of 1–3 being considered as a complete metabolic response at early interim PET. Indeed, in the succeeding Euronet-C2 trial, the threshold of radiotherapy was to be raised to DS of 4–5, instead of 3–5. The team opted to avoid radiotherapy and administer two additional (total: four) cycles of COPDAC, instead. An ultrasonography performed following six cycles of chemotherapy revealed a small cystic residual mass in the region of the left adenexa. A diagnostic laparoscopy performed to visualize and biopsy the mass demonstrated trivial bogginess of soft tissue in the pelvic wall with no visible mass. She has been well and in follow-up for 18 months.
Primary infradiaphragmatic HL has been reported in 4%–13% of patients in studies encompassing all age groups.[7,8] Published literature on isolated pelvic disease is restricted to adults.[2,3] Infradiaphragmatic HL demonstrates distinct characteristics when compared to supradiaphragmatic disease such as older age, multiple involved sites, increased frequency of lymphocyte predominant histology, and a reduced frequency of NS variants.[7,8,9,10] Survival rates are not observed to be different, and recommendations for treatment do not differ.[4,7,8,9] Bulky disease with an inadequate response on interim FDG-PET is ideally radiated, albeit with increased risk of gonadal failure, premature menopause, and infertility in pelvic HL. According to the Lugano classification for the management of lymphomas, DS-3 on interim FDG-PET indicates a favorable prognosis in most patients; interpretation must take into account timing of assessment, clinical context, and treatment. We consciously avoided radiotherapy and administered two additional cycles of chemotherapy to avoid gonadal failure. A longer follow-up is necessary for ensuring a relapse-free period.
Pelvic HL is a rarity in children. The decision for radiotherapy is arduous due to the risk of infertility.
There are no conflicts of interest.
The inputs for management from Dr. Stephen Daw (Department Paediatric and Adolescent Oncology, University College London Hospital NHS Foundation Trust, London) and Dr. Rakesh Mittal (Ex-consultant, Kuwait Cancer Center, Ministry of Health, Kuwait) are gratefully acknowledged.