A lump in the neck Lent term year 2

The case A 58 year old man went to his GP after he noticed a lump growing in the right side of his neck He had been feeling tired recently and had noticed some night sweats over the past three weeks He had also noticed some itching of his skin all over his body

What can cause lumps in the neck?

Lumps in the neck Congenital or acquired Think of organs within the neck Skin: lipoma, epidermoid cyst Congenital rests: thyroglossal cyst; branchial cysts Thyroid: cysts, goitre, tumour: adenoma or carcinoma Pharyngeal pouch Salivary gland swelling; vascular swelling Lymph nodes

Causes of lymphadenopathy Viral: “Glandular fever”EBV; CMV; HIV Bacterial: draining area of sepsis Tuberculosis; brucellosis Collagen disease Sarcoidosis Metastatic cancer e.g stomach; head & neck Lymphoma

Lymphoma: a histological diagnosis Hodgkin’s disease (20%). Reed-Sternberg cells Non-Hodgkin’s disease (80%): over 20 types B- cell lymphomas T- cell lymphomas Others: AIDS-related and CNS lymphoma

Classification of NHL: Anaplastic large cell Burkitt Cutaneous T-cell Diffuse large B-cell Follicular Lymphoblastic MALT Mantle cell Mediastinal large B-cell Nodal marginal zone B-cell Peripheral T-cell Small lymphocytic Thyroid Waldenstrom's macroglobulinaemia

Investigations: diagnosis and staging Histological biopsy Chest X-ray CT/MRI/PET scanning Liver function tests Full blood count ? Bone marrow sampling

Histology: Diffuse B Cell Lymphoma (High Grade)

PET scanning: Positron Emission Tomography

Staging of NHL: Stage 1: involving one lymph node group Stage 2: more than one group on one side of diaphragm Stage 3: involvement of lymph nodes both sides of diaphragm Stage 4: extralymphatic spread: liver, spleen, lungs, stomach, bone marrow

What sort of treatment will our patient be offered, and what is the prognosis?

Treatment for stage 4 high-grade NHL Depends on –histological type, age, general health, other organ involvement An MDT approach Will require patient’s consent after discussing type of treatment, advantages, disadvantages, risks, side effects and other options

International Prognostic Index: 2 or more factors <50% relapse-free at 5 years Age >60 years of age Serum lactate dehydrogenase-LDH elevated Performance status (0 or 1 versus 2-4), Stage III or IV Extranodal site involvement

Performance status Grade Description Fully active, able to carry on all pre-disease performance without restriction 1 Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature, e.g., light house work, office work 2 Ambulatory and capable of all selfcare but unable to carry out any work activities. Up and about more than 50% of waking hours 3 Capable of only limited selfcare, confined to bed or chair more than 50% of waking hours 4 Completely disabled. Cannot carry on any selfcare. Totally confined to bed or chair 5 Dead

Overall survival Risk Group Number of Factors % 5 year OS % 10 year OS Low 0-1 90.6 70.7 Intermediate 2 77.6 50.9 High 3 or higher 52.5 35.5

Our patient was offered chemotherapy with Ritoximab (R-CHOP) R- Ritoximab IV C- cyclophosphamide IV H- Doxorubicin (red) IV O- Vincristine IV P- Prednisolone orally 60-80mg 5 days 6-8 cycles over 21 days

Ritoximab Monoclonal Ab Locks onto protein CD20 on B lymphocytes Given as IV infusion over a few hours May insert PICC line

What side effects would you warn him about?

Side effects (variable) During infusion: ‘flu-like symptoms, flushing, allergic reactions Reduced resistance to infection (neutropenia) Bruising/bleeding (thrombocytopenia) Anaemia Fatigue Nausea and vomiting

And more… Hair loss Stomatitis Altered taste (dysguesia) Dyspepsia and hunger (steroids) Cystitis (cyclophosphamide) Numbness/tingling of fingers (vincristine) Oedema (prednisolone) photosensitivity

And more… Mood swings (prednisolone) Nail changes Cardiac arrhythmias (doxorubicin cardiomyopathy) Tissue necrosis as venous infusion site Raised blood sugar (prednisolone) Cushing’s syndrome (prednisolone)