Trachea - Hodgkin Lymphoma - Erler Zimmer
Clinical History
A 45-year-old male presented with a lump in his left supraclavicular area that had been progressively growing over a 6-month period. An excision biopsy revealed Hodgkin lymphoma (HL). Ten months later, he was readmitted with left shoulder pain and swelling in his left arm. Examination disclosed generalized lymphadenopathy, with significant swelling in the left supraclavicular and axillary regions. He underwent treatment with radiotherapy and Thiotepa chemotherapy but developed vomiting. A subsequent barium meal revealed duodenal obstruction from extrinsic lymph node compression. Unfortunately, his condition continued to deteriorate, and he passed away 2 weeks after readmission.
Pathology
The 3D print displays the tracheal bifurcation with adjacent para-tracheal and peri-bronchial lymph nodes. The trachea, opened longitudinally and viewed from behind, shows pale and matted (fused) para-tracheal lymph nodes. A confluent pale mass is evident on the left side of the trachea, above the aortic arch, which is cross-sectioned as a void space with arising branches. Enlarged peri-bronchial lymph nodes contain carbon pigment. Circumscribed paler areas in the lymph nodes and extra-nodal tumor represent foci of necrosis. An atheroma is present in the aorta wall, although challenging to visualize in the 3D print.
Further Information
Hodgkin Lymphoma (HL), a malignancy of lymphocytes, is characterized by neoplastic giant cells called Reed-Sternberg cells. There are five main subtypes based on the WHO Lymphoma Classification, distinguished by morphology, immunophenotyping, and genetics. Activation of the NF-kB transcription factor is a common tumorigenesis pathway across subtypes, promoting proliferation, reducing apoptosis, and inducing cytokine expression that recruits immune cells surrounding Reed-Sternberg cells in HL.
HL exhibits a bimodal age distribution, peaking in late adolescence/early adulthood and again in older adults, constituting just under 1% of all global cancers. Epstein-Barr Virus (EBV) infection contributes to the pathogenesis of HL subtypes. EBV induces genetic alterations affecting signaling pathways, although the exact mechanism is not fully understood. Risk factors include immunosuppression (e.g., HIV or post-organ transplant) and a positive family history. Common HL presentations include painless lymphadenopathy, pruritus, weight loss, fevers, and night sweats. Advanced disease may involve organ spread to the spleen, liver, and bone marrow, causing compressive symptoms. Diagnosis involves staging CT scans, excision biopsy of involved nodes, and bone marrow biopsy. Treatment includes radiotherapy and chemotherapy, leading to significant improvements in HL survival rates over the last five decades.
