Neoplasia VI

• Malignancy in lymph nodes can be due to:

  1. Metastatic tumor:

  • Carcinoma (common)
  • Melanoma
  • Sarcoma (rare)
  • Other rare tumors like Germ cell

  2. Haematological malignancy, such as lymphoma
• Clinical manifestations of Lymphoma include:

  • Painless lymphadenopathy
  • Abdominal distention, ascites
  • Shortness of breath, pleural effusion
  • Fatigue
  • Fever
  • Night sweats
  • Weight loss
  • Neurological symptoms in CNS disease
• Ann Arbor Staging for lymphoma:

  • I: single lymph node region
  • II: One side of diaphragm
  • III: Both sides of diaphragm
  • IV: Disseminated
  • A: No systemic symptoms
  • B: Fever, night sweats, weight loss

  Imaging modalities in Lymphoma include:

  • CXR
  • CT
  • MRI
  • PET
  • Bone Aspirate
  • Bone Trephine
  • LDH level
  • Performance index
• Histopathological diagnosis methods for lymphoma:

  • Fine needle biopsy
  • Trucut biopsy
  • Incisional biopsy
  • Excision of whole lymph node

  The best method for the pathologist is excision of the whole lymph node because it provides a more comprehensive view
• Lymphomas are monoclonal, meaning they are derived from a single ancestral cell by repeated cellular replication. In malignancy, these cells divide unchecked due to cell cycle dysregulation, forming a single clone. V(D)J recombination is the mechanism of somatic recombination that occurs in developing lymphocytes, resulting in a diverse repertoire of antibodies and T cell receptors
• Classification of lymphomas is important because it:

  • Provides diagnostic criteria
  • Allows correct treatment
  • Provides prognostic data
  • Allows comparison of treatment trials

  The classification should be reproducible
• Classification of NHL includes:

  • Indolent
  • Aggressive
  • Nodular (follicular)
  • Diffuse
  • Small cell
  • Large cell
• Hodgkin Lymphoma features:

  • Classical Hodgkin
  • Nodular sclerosis
  • Mixed cellularity
  • Lymphocyte-rich classical
  • Lymphocyte-depleted
  • Nodular lymphocyte-predominant Hodgkin
• Non-Hodgkin Lymphoma types:

  • B-cell:
  • High-Grade: Burkitt lymphoma, Diffuse large B-cell lymphoma, Mantle cell lymphoma
  • Low-Grade: Follicular, Marginal zone lymphoma, Lymphoplasmacytic lymphoma
  • T-cell:
  • Most behave as high-grade
  • Peripheral T-cell Lymphoma, Angioimmunoblastic T-cell lymphoma, Anaplastic large cell lymphoma, Enteropathy-associated T-cell NHL, Mycosis fungoides, Sezary syndrome
• Burkitt Lymphoma:

  • Presents with a "starry sky" appearance on histological section of marrow
  • Requires aggressive chemotherapy
  • Main forms: sporadic, endemic, HIV-associated
  • MYC translocation t(8:14)
• Mantle Cell Lymphoma:

  • Low-grade B-cell lymphoma
  • <5% of lymphomas
  • Peak incidence at 60-70 years
  • Often high stage at presentation
  • Cyclin D1 positive
  • 20-40% 5-year survival despite being low grade
• Follicular Lymphoma:

  • Low-grade
  • Common lymphoma
  • Age 50+
  • 60% stage III or IV at presentation
  • Rarely cured, 20% transform to high grade
  • BCL2 positive
• T Cell Lymphoma:

  • Low Grade: Mycosis fungoides, Sezary syndrome
  • High Grade: Peripheral T-cell lymphoma, Enteropathy-associated T-cell lymphoma, T lymphoblastic lymphoma, Adult T-cell leukaemia/lymphoma
• Mycosis Fungoides and Sezary syndrome:

  • Both cutaneous T cell lymphomas
  • Therapeutic aim is to control disease, reduce symptoms, and improve cosmetics
  • Allogenic bone marrow transplantation is the only curative treatment for a fraction of patients with advanced disease
• Adult T Cell Leukaemia/Lymphoma:

  • Rare but more common in Japan and the Caribbean
  • HTLV-1 transmission through infected cells in breast milk, semen, and blood
  • Infection established by cell-to-cell contact
  • Genetic/epigenetic aberrations accumulate, leading to ATL