TUMOURS AND HIV Flashcards by

What are the three AIDS defining cancers?

Kaposi sarcoma

High grade B-cell non-Hodgkin’s lymphoma

Invasive cervical cancer

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How much more common are high grade B-cell non-Hodgkin’s lymphomas (NHL) in patients who are HIV positive versus the HIV negative population?

60 - 100 times more common

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What are the main types of high-grade B-cell non-Hodgkin’s lymphomas (NHL) found in patients who are HIV positive?

2/3rds are diffuse large cell lymphomas

1/3rd are Burkitt or Burkitt like lymphomas

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What are the clinical features of systemic (as opposed to cerebral) non-Hodgkin’s lymphoma (NHL)?

Lymph nodal disease (50%)

GI disease (30%)

Extra-nodal disease (20%)

Effusions without nodal masses - primary effusion lymphoma (1%)

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What is primary effusion lymphoma (effusions without nodal masses) associated with?

Kaposi sarcoma herpes virus

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How do we treat AIDS-related systemic non-Hodgkin’s lymphomas?

Combination anthracycline-based chemotherapy with intrathecal chemotherapy for those at risk of meningeal relapse.

Given with continuing cART (combined anti-retroviral therapy)

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What percentage of patients with AIDS-related systemic non-Hodgkin’s lymphomas will achieve durable complete remission?

50-60%

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What is primary cerebral lymphoma?

Non-Hodgkin’s lymphoma that is confined to the craniospinal axis without systemic involvement.

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Why has the incidence of primary cerebral lymphoma decreased dramatically?

Rates of PCL have been affected by the introduction of cART more than rates of systemic NHL

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What are the two most common causes of cerebral mass lesions in people with HIV?

Primary cerebral lymphoma

Toxoplasmosis

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What are the clinical features of primary cerebral lymphoma?

Headaches

Focal neurological deficits

Very low CD4 count (less than 50 cells/microlitre)

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How might the history help you differentiate primary cerebral lymphoma from toxoplasmosis?

Gradual onset over 2-8 weeks

Absence of fever

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What investigations would you do in someone you suspected primary cerebral lymphoma?

CT or MRI

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What do CT or MRI show in a patient with primary cerebral lymphoma?

Solitary or multiple ring enhancing lesions with prominent mass effect and oedema.

NB, these features also occur in toxoplasmosis

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How might MRI or CT help you differentiate primary cerebral lymphoma from toxoplasmosis?

Look very similar

PCL lesions usually periventricular

Toxoplasmosis more often affects the basal ganglia

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How do you initially treat someone who is HIV positive and severely immunodeficient, and is diagnosed with a cerebral lesion?

Assume toxoplasmosis until they do not respond clinically and radiologically for 2 weeks of anti-toxoplasma therapy.

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What should you look for in the CSF of all patients in whom you suspect primary cerebral lymphoma?

EBV DNA

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What is the investigation that can help differentiate between primary cerebral lymphoma and cerebral toxoplasmosis?

18-Flurodeoxyglucose positron emission tomography (FDG-PET)

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How do we manage patients with confirmed primary cerebral lymphoma?

Whole brain irradiation

Commence cART in those who anti-retroviral naive

Some clinicians advocate high dose IV chemotherapy

What is the overall survival period of primary cerebral lymphoma?

Median survival 1-3 months

What is the microorganism associated with Kaposi sarcoma?

Kaposi sarcoma herpes virus (KSHV)

Human herpes virus 8 (HHV8)

What is the possible routes of transmission of human herpes virus 8 (Kaposi sarcoma herpes virus)?

Saliva (kissing)

Also transmitted vertically

What is the histological pattern of Kaposi sarcoma?

Proliferation of spindle-shaped cells accompanied by endothelial cells, fibroblasts and inflammatory cells that form slit-like vascular channels that resemble neo-angiogenesis.

What are the typical lesions associated with Kaposi sarcoma?

Multiple

Pigmented

Raised

Painless

Not blanching

Usually follow the line of skin creases

May develop to form large plaques and nodules

What are the earliest cutaneous lesions associated with Kaposi sarcoma?

Innocuous looking macular-papular pigmented lesions

Other than the skin, where should you look for lesions of Kaposi sarcoma in a patient who is HIV positive?

Oral cavity Conjunctiva

What is the type of Kaposi sarcoma more commonly seen in people of African descent that is often particularly difficult to control?

Nodular KS Often associated with lymphoedema

What are the most common visceral sites associated with Kaposi's sarcoma?

Lungs Stomach

What are the features of pulmonary Kaposi's sarcoma?

Dyspnoea Dry cough With or without fever May cause haemoptysis

How do we usually manage someone with KS unless it isT1 staged?

cART alone - most patients will have responded within 6-12 months Lesions may be removed for cosmetic reasons

What are the first line chemotherapy agents used in the management of Kaposi sarcoma that has progressed to a T1 stage?

Liposomal anthracyclines

What is the prognosis of Kaposi sarcoma?

Usually very good unless it has progressed to pulmonary KS (median survival of 18 months)

How does HIV increase risk of cervical cancer?

Higher prevalence of HPV in cervix Higher frequency of multiple HPV genotypes Persistence of HPV in cervix Higher rate of progression from low grade squamous intraepithelial lesion (SIL) to high grade SIL Higher likelihood of relapse of CIN II/III after therapy

In which HIV positive women is the risk of squamous intraepithelial lesions (SIL) the highest?

Those with a CD4 count below 200 cells/microlitre

How does the treatment of cervical cancer in HIV positive women differ from those who are HIV negative?

Start cART Otherwise the same

What are the non-AIDS defining but HIV-related cancers?

Anal cancer or anal intraepithelial dysplasia Lung cancer Seminoma Head and neck cancer - associated with HPV Hodgkin's lymphoma Castleman's disease (giant lymph nodes - lymphoproliferative disorder)

Why is the incidence of anal cancer higher in the HIV positive population?

Association with MSM Association with HPV

What are the clinical features of anal cancer?

Pain Bleeding Mass

How do we manage HIV related anal cancer?

Combined modality therapy of chemotherapy and radiation treatment with sphincter preservation.

What is the 5 year survival rate of HIV related anal cancer?

65%

What is the incidence of Hodgkins lymphoma in the HIV positive population compared with the HIV negative population?

10 times higher

What is the virus implicated in the pathogenesis of HIV related Hodgkin's lymphoma?

EBV

What are the clinical features of HIV related Hodgkin's lymphoma?

B symptoms (fever, weight loss, night sweats) Bone marrow infiltration Extranodal disease

What is multicentric Castleman's disease?

A relatively rare lymphoproliferative disorder. It is related to KSHV.

What are the diagnosis criteria of HIV associated multicentric Castleman's disease?

Fever with at least 3 of the following: Peripheral lymphadenopathy Hepatosplenomegaly Oedema Pleural effusion Ascites Cough Nasal obstruction Xerostomia Rash Central neurological symptoms Jaundice Autoimmune haemolytic anaemia

How do we treat HIV associated multicentric Castleman's disease?

Rituximab in combination with cART

What is the prognosis of multicentric Castleman's disease?

Good