Dr Theresa Raju, Dr Rema, Dr Praveena, Dr Jayakumar, AIMS Kochi
Fourteen years old boy apparently normal 3 weeks back presented high grade intermittent fever of 103F. Nocturnal fever was associated with night sweats and tiredness .Initially he was managed as case of viral fever and subsequently for UTI as urine showed pyuria
USG done was normal.
Since fever persisted Irrespective of treatment and the occasional cough the child developed, a Chest xray was taken which showed prominence of anterior mediastinum
CT chest taken due to this showed anterior to aortic arch and pre vascular region enhancing lobulated confluent nodular soft tissue mass(9.1 X 6.5 X 8.2cms) with necrotic areas
Diffrentials considered were
1. Infections like tuberculosis
2EBV
2. Malignancies like Hodgkins Lymphoma , Leukemia
3. Miscelleneous:
4 Teratoma
5 Neuro blstoma
No history of breathing difficulty, chest retractions, documented weight loss, loss of appetite, burning micturition, increased frequency/urgency of micturition, vomiting, travel, contact/exposure with animals, drug intake, joint pain, photosensitivity, oral ulcers, rashes
There is history of 2 admission at nine months back for viral fever and another at 5 years for age for pneumonia
Paternal Grandfather had carcinoma liver and maternal grandmother had CA lung
At admission the child was febrile but with stable vitals
Submandibular lymph nodes was non tender and axillary and inguinal lymph nodes were tender
Head to foot examination was normal
Auxology was normal
Systemic examination -no hepatosplenomegaly.
Pl
Labs
TC:7.65Ku/ml, N:75.3%, L:12.3%, , ESR:67mm/1sthr, CRP:81.60mg/L
RFT,LFT, Serum electrolytes and pre op serology were normal
Urine and blood culture were sterile
USG abdomen done showed bilateral mild pelviectasis nooner mass or Hepato splenomegaly
USG guided targeted biopsy was taken the interventional radiologist from the anterior mediastinal mass and HPE was provisionally reported as polymorphous infiltrate with hyalinisation and was advised IHC.
IHC done showed CD3, CD20- admixture seen, CD30 -positive in large cells, PAX5- weak positive in large cell. This was diagnosed as Hodgkins Lymphoma- Nodular sclerosis type. PET-CT scan for staging and prognostication was done
PET MRI showed: FDG avid multiple supradiaphragmatic lymph nodes. Diffuse FDG uptake noted in spleen and marrow of almost entire visualised axial and appendicular skeleton-likely lymphomatous involvement. FDG PET MRI staging for lymphoma according to LUGANO’S classification shall be stage IV.
The child was initiated on OEPA regimen(Vincristine/Oncovin, Etoposide, Prednisone, Doxorubicin/Adriamycin)as per protocol.
HODGKIN’S LYMPHOMA:
It is a lymphoreticular neoplasm primarly affecting B cell lineage involving lymph nodes and the lymphatic system.
There is a male predominance in children affected below 7 years of age, with equal sex distribution beyond 12 years of age
Pathology: Lymph nodes are the most common tissue on which the diagnosis of hodgkins lymphoma is made.
The WHO classification of Hodgkins Lymphoma recognizes two subtypes- nodular lymphocytic predominant hodgkins lymphoma(NLPHL) and the commoner classical Hodgkins Lymphoma(Chl).
The main tumour mass predominantly consists of non neoplastic tumour cells. The hallmark of classic hodgkins lymphoma is Reed Sternberg(RS) cells. This is a binucleated or multinucleated that is characterised bilobed nucleus with 2 large nucleoli giving an owl eye appearance to the cells.
Clinical features: Presents with painless cervical or supraclavicular lymphadenopathy. The nodes are firm and rubbery in consistency. About 20-30% of children presents with B symptoms, as defined the Ann Arbor staging criteria
Fever> 38C,
Night sweats
Unexplained weight loss >10% body weight.
Management: Most children are treated with combination chemotherapy alone or in combination with radiotherapy. Treatment of patients with favourable clinical presentation (localized node involvement: stage I,II,IIIA; absence of B symptoms ; no evidence of bulky disease) consists of 2-4 cycles of chemotherapy (ABVD)and low dose involved field radiation.
Patient with unfavourable clinical presentation(B Symptoms, bulky mediastinal/peripheral lymphadenopathy; extranodal extension of disease; advance stage-IIIB-IV) are treated with 4-6 cycles of ABVD with or without radiotherapy.
Other combinations include OPPA(Vincristine, Procarbazine, Prednisone, Doxorubicin); OEPA(Vincristine, Etoposide, Prednisone, Doxorubicin) and BEACOPP( Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, Prednisolone)
Hodgkins Lymphoma is one of the most curable cancers of childhood, especially if detected in early stages.
Appropriate staging , availability of advanced techniques of investigations and use of risk adapted treatment protocols have resulted in excellent overall survivals.