APCON 2008 Chennai

 

NATIONAL PATHOLOGY QUIZ – 1

Answers

 

Q 1: Enteropathy associated T cell Lymphoma

The first four pictures are of the duodenum (Brunner’s glands can be seen beneath the muscularis mucosa). There is complete villous atrophy. The surface is flat and no villi are seen. There is great increase in the number of Intraepithelial lymphocytes (IELs). This appearance is typical of celiac disease. The subsequent slides show collections of somewhat monomorphic lymphoid cells in the bone marrow trephine biopsy which are shown to be T cells by IHC.

Correct answers: 68.9%

 

Links

1. Enteropathy associated T cell Lymphoma

2. Mucosal Intra-epithelial Lymphocytes

 

 

Q 2: Proteus syndrome

The still is from the movie ‘Elephant man’. Based on the life story of Joseph Merrick, the film directed by David Lynch stars John Hurt as the Elephant man and Anthony Hopkins as the surgeon who is professionally intrigued by his condition. Despite the hideous deformities and being regarded an imbecile, it is revealed that beneath the physical appearance lies an intelligent and erudite human being.

The condition that Herrick inherited was for long thought to be neurofibromatosis. However, the consensus of opinion now is that he had ‘Proteus syndrome’. Proteus syndrome is a highly variable, severe disorder of asymmetric and disproportionate overgrowth of body parts, connective tissue nevi, epidermal nevi, dysregulated adipose tissue, and vascular malformations.

Correct answers: 29.7%

 

Links

1. Proteus syndrome

2. Joseph Merrick

3. Elephant man (Movie)

 

Q 3: Papulonodular mucinosis in SLE

The skin biopsy shows great amount of stringy bluish staining material in the dermis. Occasional basal cells show hydropic change. No significant inflammation is seen. In a patient with history of suggestive history like joint pains, it is reason enough to suspect SLE which can show these lesions, also known as nodular cutaneous lupus mucinosis.

This patient indeed was proved to have SLE and the dermal material stained strongly with Alcian Blue.

Correct answers: 10.8%

 

Links

1. Papulonodular mucinosis in SLE

 

Q 4: Congenital Epulis (Granular cell tumor of the newborn)

As almost all of you have noted, it is very clearly a lesion composed of large granular cells. The overlying epithelium is not hyperplastic as is usually seen in granular cell tumors. There is an island of odontogenic epithelium, but the granular cells are not seen within this epithelium, but lying outside in a diffuse fashion. So, it is not a granular cell variant of ameloblastoma – which incidentally was the diagnosis most of you preferred.

Finding of these islands of odontogenic epithelium and the absence of epithelial hyperplasia are characteristic features of congenital granular cell epulis.

This was from a newborn female baby. But, giving that history would have been a give-away!

Correct answers: 17.6%

 

Links

1. Congenital epulis

 

Q 5: Budd-Chiari syndrome in Sickle cell anemia

The liver shows intense congestion and hemorrhage sharply confined to the centizonal region. The periportal regions remain unaffected. In these areas the sinusoids are dilated and contain erythrocytes that are elongated, many being sickle shaped.

This was postmortem liver tissue fro a 29 year old woman with sickle cell anemia. There was a thrombus occluding the hepatic vein.

Budd-Chiari syndrome is one of the rarer complications of sickle cell disease.

(Diagnosis of Sickle cell anemia will fetch half credit).

Correct answers: 10.8%

 

Links:

1. Budd-Chiari syndrome – A review

2. Budd-Chiari syndrome in Sickle cell disease

 

Q 6: Adrenal Myelolipoma

Lipomatous tumor containing reddish (blood containing) areas. Real clue to the diagnosis is the thin rim of adrenal tissue in the 10-11 O’Clock position (Missing it is perfectly excusable).

Histologically it was a typical Myelolipoma. The marrow elements were seen mostly in the reddish areas.

Correct answers: 17%

 

Links:

Myelolipoma CT and Pathology