Intracranial disease in pediatric Hodgkin lymphoma-case report and review of literature.

Am J Blood Res 2023;13(5):162-167 www.AJBlood.us /ISSN:2160-1992/AJBR0151072 Case Report Intracranial disease in pediatric Hodgkin lymphoma-case report and review of literature Nidhi Dhariwal1,2, Nirmalya Roy Moulik1,2, Vasudeva Bhat2,3, Vasundhara Smriti2,4, Sangeeta Kakoti2,5, Sayak Choudhury2,6, Epari Sridhar2,7, Sumeet Gujral2,7, Chetan Dhamne1,2, Sneha Shah2,6, Gaurav Narula1,2, Shripad Banavali1,2 Division of Pediatric Oncology, Department of Medical Oncology, Tata Memorial Hospital, Mumbai 400012, Maharashtra, India; 2Homi Bhabha National Institute, Anushakti Nagar, Mumbai 400094, Maharashtra, India; 3 Department of Pediatric Hematology and Oncology, Manipal Academy of Higher Education, Manipal 576104, Karnataka, India; 4Department of Radiodiagnosis, Tata Memorial Hospital, Mumbai 400012, Maharashtra, India; 5 Department of Radiotherapy, Tata Memorial Hospital, Mumbai 400012, Maharashtra, India; 6Department of Nuclear Medicine, Tata Memorial Hospital, Mumbai 400012, Maharashtra, India; 7Department of Pathology, Tata Memorial Hospital, Mumbai 400012, Maharashtra, India 1 Received May 5, 2023; Accepted August 31, 2023; Epub October 15, 2023; Published October 30, 2023 Abstract: Central nervous system (CNS) involvement in Hodgkin lymphoma (HL) is an extremely rare presentation with dismal outcomes according to reported literature. An 8-year-old girl presented to us with complaints of on-off fever, right cervical swelling and bilateral ptosis. Positron emission tomography (PET) showed intracranial extraaxial soft tissue masses in right infero-lateral temporal lobe, sella and bilateral parasellar region along with cervical, mediastinal, axillary, abdominal and inguino-pelvic nodes, liver lesions and extensive marrow lesions involving the axial and appendicular skeleton. Histopathology of the cervical lymph node revealed a diagnosis of classical Hodgkin lymphoma. Child received 2 cycles of OEPA and 4 cycles of COPP followed by radiotherapy to bulky cervical lymph nodes and intracranial lesion. The child has been disease-free for 44 months with no neurological sequalae. Intracranial spread is rare in Hodgkin lymphoma and is associated with inferior outcomes. Due to its rarity, there are no specific treatment guidelines for this entity. The choice of ideal chemotherapeutic agents and role of whole-brain radiotherapy needs further evaluation. Keywords: Hodgkin lymphoma, central nervous system, intracranial, pediatric Case progressive right neck swelling for 3 months. Physical examination revealed a firm right cervical lymph nodal mass with no other peripheral lymphadenopathy or hepatosplenomegaly. On neurological assessment, she had bilateral ptosis with mid-dilated pupils and sluggish bilateral pupillary reaction. Her baseline complete blood count showed a haemoglobin of 10.4 g/ dl, total leucocyte counts of 15.16*109/L and platelet count of 714*109/L. Baseline LDH was 391 U/L (Normal range - 110-295 U/L), and renal function tests and liver function tests were within normal limit with no evidence of tumour lysis syndrome. An 8-year-old girl presented to our outpatient department with complaints of on and off undocumented fever for 1 year and gradually A contrast-enhanced computed tomography (CECT) was done for evaluation of the ptosis which revealed extra-axial lobulated soft tissue Introduction Central nervous system (CNS) involvement in Hodgkin lymphoma (HL) is an extremely rare presentation with dismal outcomes according to reported literature. Management includes a combination of chemotherapy and radiotherapy though no standard treatment guidelines exist. We herein describe our experience with an 8-year-old girl with HL who presented with CNS involvement in addition to generalized lymphomatous involvement. Intracranial disease in Hodgkin lymphoma lesions was non-Hodgkin lymphoma (NHL), though duration of symptoms did not support the same. Hence, the child received prophase COP (Cyclophosphamide, Vincristine and Prednisolone) with dexamethasone in place of prednisolone while awaiting histopathology report and a bone marrow aspiration along with biopsy and lumbar puncture with intrathecal cytarabine administration, was done, considering an initial possibility of NHL. Histopathological examination of the cervical lymph node revealed large, atypical cells which were positive for CD30, GATA3 and weakly positive for PAX-5, while being negative for CD15, EBV-LMP1, ALK-1, CD1a and CD20, with preponderance of T-lymphoid cells in the background, suggestive of Hodgkin lymphoma. Staging PET CECT showed multiple FDG (fluorodeoxyglucose) avid homogenously enhancing solid intracranial extra-axial dura-based lesions along the right infero-lateral Figure 1. Baseline CECT of the brain showing dura-based soft lesions with temporal lobe, the sella, bilatcomplete resolution in end-of-treatment MRI in (A) axial and (B) coronal seceral parasellar region along tion. the cavernous sinuses and posteriorly along the occipital masses in bilateral parasellar region involving lobe with maximum SUV (standardized uptake the dura matter and the cavernous sinuses, values) of 10.99. Contiguous medullary involveand encasing the cavernous segment of bilatment of the bones of skull base was noted eral internal carotid arteries (Figure 1). The along with extramedullary extension into the mass indented both the temporal lobes with no right masticator space involving the temporalis obvious infiltration or oedema of the brain muscle and bilateral lateral pterygoids. The parenchyma. Superiorly it extended into the scan also showed FDG avid nasopharyngeal sella and along the pituitary stalk. Posteriorly thickening, multiple FDG avid bulky cervical the mass extended into the prepontine cisnodes (6.7*4.4*3.4 cm) along with mediastitern along the clivus. A similar soft tissue was nal, axillary, abdominal, inguinal and pelvic seen posterior to the Torcula of Herophili. The nodes, liver lesions and extensive marrow mass appeared mildly hyperdense on nonlesions involving the axial and appendicular enhanced CT scan and showed homogeneous skeleton, indicating disseminated lymphoenhancement after administration of intravematous disease with intracranial extra-axial nous contrast. involvement (Figure 2). The most probable differential diagnosis for a cervical lymph nodal mass with intracranial 163 Bone marrow aspirate and cerebrospinal fluid were uninvolved on morphology, but bone marAm J Blood Res 2023;13(5):162-167 Intracranial disease in Hodgkin lymphoma Our institutional guidelines mandate irradiation of bulky sites (peripheral lymph node with size ≥6 cm in any dimension). Hence, after completion of chemotherapy, patient received adjuvant image-guided intensity modulated radiation therapy (IG-IMRT) to the prechemotherapy extent of the intracranial lesion and right hemi-neck to a dose of 25.2 Gy in 14 fractions. She has been disease-free for 44 months now with no residual neurological deficit and withou (...truncated)