Dataset Information

Bulky Pelvic Hodgkin Lymphoma in a Prepubertal Girl.

ABSTRACT: 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.

SUBMITTER: Totadri S

PROVIDER: S-EPMC5582567 | biostudies-other | 2017 Apr-Jun

REPOSITORIES: biostudies-other

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Bulky Pelvic Hodgkin Lymphoma in a Prepubertal Girl.

Totadri Sidharth S Bansal Deepak D Bal Amanjit A Menon Prema P

Indian journal of medical and paediatric oncology : official journal of Indian Society of Medical & Paediatric Oncology 20170401 2

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-fluorodeoxyg ...[more]

PMID: 28900338

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Project description:PurposePatients with bulky stage I/II classic Hodgkin lymphoma (cHL) are typically treated with chemotherapy followed by radiation. Late effects associated with radiotherapy include increased risk of second cancer and cardiovascular disease. We tested a positron emission tomography (PET)-adapted approach in patients with bulky, early-stage cHL, omitting radiotherapy in patients with interim PET-negative (PET-) disease and intensifying treatment in patients with PET-positive (PET+) disease.MethodsEligible patients with bulky disease (mass > 10 cm or 1/3 the maximum intrathoracic diameter on chest x-ray) received two cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) followed by interim fluorodeoxyglucose PET (PET2). Patients with PET2-, defined as 1-3 on the 5-point scale, received four additional cycles of doxorubicin, bleomycin, vinblastine, and dacarbazine. Patients with PET2+ received four cycles of escalated bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone followed by 30.6 Gy involved-field radiation.ResultsOf 94 evaluable patients, 53% were female with median age 30 years (range, 18-58 years). Eight-five (90%) had stage II disease, including 48 (51%) with stage IIB/IIBE. Seventy-eight (78%) were PET2- and 21 (22%) were PET2+. The predominant toxicity was neutropenia, with 9% of patients developing febrile neutropenia and one developing sepsis. The primary end point of 3-year progression-free survival (PFS) was 93.1% in PET2- and 89.7% in PET2+ patients. Three-year overall survival was 98.6% and 94.4%, respectively. The estimated hazard ratio comparing PFS of patients with PET2+ and patients with PET2- was 1.03 (85% upper bound 2.38) and was significantly less than the null hypothesis of 4.1 (one-sided P = .04).ConclusionOur study of PET-adapted therapy in bulky stage I/II cHL met its primary goal and was associated with an excellent 3-year PFS rate of 92.3% in all patients, with the majority being spared radiotherapy and exposure to intensified chemotherapy.

Dataset Information

Bulky Pelvic Hodgkin Lymphoma in a Prepubertal Girl.

Publications

Bulky Pelvic Hodgkin Lymphoma in a Prepubertal Girl.

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