AIDS-related Kaposi sarcoma

Case contributed by Dr Rayyan Mardhiyah

Presentation

A retroviral disease patient presented with a two month history of fever, cough and loss of weight and appetite. Multiple purple brown papules noted over head, scalp, neck, and trunk.

Patient Data

Age: 30 years
Gender: Male
X-ray

Chest radiograph

Frontal chest radiograph showed reticular opacities and multiple parenchyma lung nodules.

X-ray

Chest radiograph

There is worsening reticular opacities in perihilar distributions and increasing size of the multiple lung nodules over a two month period -- initially treated as pneumocystis jiroveci pneumonia (PJP).

CT

CT chest, abdomen, and pelvis

Thorax:

  • multiple scattered lung nodules in a peribronchovascular subpleural distribution, with  flame shape appearance of the lung nodules
  • bilateral enhancing axillary lymph nodes and enhancing subcutaneous nodules

Abdomen & Pelvis:

  • multiple small hypoattenuating liver and splenic nodules
  • multiple enhancing peritoneal nodules
  • enhancing bilateral inguinal nodules
  • multiple lytic lesions of the visualized bones

Case Discussion

Histopathologic examination confirmed Kaposi Sarcoma

This patient has disseminated Kaposi Sarcoma (skin, lung, visceral and gut). The patient developed persistent episodes of diarrhea and a colonoscopy revealed multiple erythematous fungating lesions, extending from the rectum to cecum. After the patient started chemotherapy his condition improved, however after few months he succumbed to severe metabolic acidosis and hyperkalemia with multiple organ dysfunction.

There are four variants of Kaposi Sarcoma:

  • classic form
  • endemic form (african)
  • iatrogenic (organ transplant related)
  • AIDS-related Kaposi Sarcoma

with the two latter form being the most common and disseminated form 1

Kaposi Sarcoma is caused by Human Herpes Virus type 8 1.

AIDS-related Kaposi sarcoma is highly associated with patient who are male and homosexual and virus usually transmitted from the anal sexual intercourse 1

In this disseminated form, commonly involved organs include

1) Head & neck

  • cutaneous form with skin, face and neck nodules 1
  • mucosal form ( intra-oral, laryngeal and pharyngeal nodules 1

2)  Thoracic                        

CT has certain characteristic findings compared to chest radiograph with flame shape appearance of lung nodules (bilateral symmetrical ill defined nodules in peribronchovascular distributions 1). 

These nodules may presented with "halo" sign: ground glass opacities surrounding the nodules. Other associated findings include peribronchovascular and interlobular septal thickening, fissural nodularity, mediastinal, hilar and axillary adenopathy 1.

3) Liver & Spleen

Multiple hypo-attenuating nodules. Nodules may exhibit delayed enhancement distinguishing it from multiple hemangiomas 1. Lesions should be hyperechoic on ultrasound scan. 

4) Gastrointestinal tract

Can affect gastrointestinal tract from oropharynx to the rectum. Biopsy may be negative as it is a submucosal lesion 1.

In this case, with the abdominal findings, other differential diagnoses that were considered included fungal lesions in view of the liver and spleen lesions with concurrent intra-abdominal TB. He was treated for opportunistic infection with fungal (amphotericin) and challenge with TB treatment before he was subjected for anti-retroviral therapy (ART) and chemotherapy. The initial chest radiograph was confused with PJP but the CT findings were characteristic for KS with absence of pleural effusion which may be present in PJP.

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Case information

rID: 85247
Published: 1st Jan 2021
Last edited: 1st Jan 2021
System: Oncology
Inclusion in quiz mode: Included
Institution: Kkm