Case Number : Case 122 Posted By: Guest

Phillip McKee - Overseas consultations (Sedona, Arizona, USA) Wrote:

This is a very difficult case of a very rare condition in which the cutaneous lesions are more often than not non-specific (at least in the few cases that I have personally seen). The diagnosis is Castleman’s disease. Castleman’s disease (angiofollicular lymph node hyperplasia) typically affects lymph nodes and is a B-cell proliferative disorder which can be associated with systemic symptoms. It may be associated with HIV/AIDS & HHV8. Interleukin 6 (IL6) plays an important role in the pathogenesis of the disease. Two variants- hyaline vascular and plasma cell types are recognized histologically. Cutaneous lesions are exceptionally rare. The cutaneous lesions when prominent as large nodules are characterized by dense infiltrate of lymphocytes and plasma cells (polyclonal) associated with a prominent increased vascularity. The absence of cytological atypia and prominent vessels are pointers to the correct diagnosis. Rarely, the hyaline vascular variant is encountered in the skin with dense fibrosis associated with germinal centers and a conspicuous mantle zone. It is however not possible to make a definitive diagnosis is this case. Rich Carr sent me the case and like all things exotic, it proved impossible for me not to include an image. Angioimmunoblastic T-cell lymphoma is a good differential diagnosis as there is certainly prominence of the blood vessels but the lymphocytic atypia is absent. B-cell pseudolymphoma would also be a good differential diagnosis.

Submitted on 26/11/2010 19:01
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Mona Abdel-Halim - Dermatology Department, Faculty of Medicine, Cairo University (Egypt) Wrote:

Hi all. The valuable comment of Dr Juan Carlos has triggered me to look in the lymphoma area. Lymphoma is also one of the important DDx of small blue cell neoplasms. I think I came up with a nice idea, could this be Lennert's lymphoma (lymphoepithelioid TCL), in which we see clusters of epithelioid histiocytes admixed with the lymphocytes??? I love these challenging cases!!!

Submitted on 26/11/2010 16:50
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Miah Singh - (UK) Wrote:

Difficult case - lymphoma of some sort!

Submitted on 26/11/2010 15:15
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Carlos B - (Spain) Wrote:

I also agree with 'high endothelial cells' and therefore T cell lymphoma

Submitted on 26/11/2010 15:14
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Juan Carlos Garcés - Hospital Oncológico / Hospital Luis Vernaza (Guayaquil Ecuador) Wrote:

Good morning everyone.. I am not sure of anything in this case.. Those long branching estructures remind me postcapillary venules with very plump endothelial cells (however they could be epithelial!) in a rich lymphocyte background. I am thinking in Lymphoma..(angioimmunoblastic? ... I dont see polymorphus infiltrate )(peripheral T cell lymphoma?). Sorry if I am very wrong..I am wainting very anxiously other opinions and the rigth answer. Great weekend to everyone in all the continentes!!

Submitted on 26/11/2010 14:12
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Eman El-Nabarawy - Cairo University (Egypt) Wrote:

Difficult case. I thought of Merckle cell carcinoma with squamous diffrentiation.

Submitted on 26/11/2010 12:21
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Richard Carr - Warwick (UK) Wrote:

Dear Phillip and colleagues. Posted some comments on yesterday's case. Beutiful cold day with blue skies in Warwick today - I have stopped believing in global warming. Sincerely hoping we will defeat the boks at Rugby and Aussies at criket this weekend!! What joy to be alive. Regards to all, Richard

Submitted on 26/11/2010 10:02
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Mona Abdl-Halim - Dermatology Department, Faculty of Medicine, Cairo University (Egypt) Wrote:

Sorry for the spelling mistake, I meant to say prominent nucleoli.

Submitted on 26/11/2010 09:32
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Mona Abdel-Halim - Dermatology Department, Faculty of Medicine, Cairo University (Egypt) Wrote:

Very difficult case. I will give it a try. Solid sheets of small atypical blue cells. Larger cells are sprinkled about the blue cells containing rounded nuclei, prominent nuclei and I am not sure but I think marginated chromatin is seen in some of them. Could this be a neuroblastoma with neuronal differentiation??? Could not identify clearly neuropoil formation, may be it is not that well differentiated. Or may be some other primitive neuroectodermal tumor. Looking forward to know what this case is!!!

Submitted on 26/11/2010 09:30
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Phillip H. McKee - Overseas consultations (Arizona, USA) Wrote:

The case was kindly shared with me by Dr Richard Carr, from the U.K.

Submitted on 25/11/2010 23:05
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Phillip McKee - Overseas consultations (Sedona, Arizona, USA) Wrote:

This is a very difficult case of a very rare condition in which the cutaneous lesions are more often than not non-specific (at least in the few cases that I have personally seen). The diagnosis is Castleman’s disease. Castleman’s disease (angiofollicular lymph node hyperplasia) typically affects lymph nodes and is a B-cell proliferative disorder which can be associated with systemic symptoms. It may be associated with HIV/AIDS & HHV8. Interleukin 6 (IL6) plays an important role in the pathogenesis of the disease. Two variants- hyaline vascular and plasma cell types are recognized histologically. Cutaneous lesions are exceptionally rare. The cutaneous lesions when prominent as large nodules are characterized by dense infiltrate of lymphocytes and plasma cells (polyclonal) associated with a prominent increased vascularity. The absence of cytological atypia and prominent vessels are pointers to the correct diagnosis. Rarely, the hyaline vascular variant is encountered in the skin with dense fibrosis associated with germinal centers and a conspicuous mantle zone. It is however not possible to make a definitive diagnosis is this case. Rich Carr sent me the case and like all things exotic, it proved impossible for me not to include an image. Angioimmunoblastic T-cell lymphoma is a good differential diagnosis as there is certainly prominence of the blood vessels but the lymphocytic atypia is absent. B-cell pseudolymphoma would also be a good differential diagnosis.

Submitted on 26/11/2010 19:01
________________________________________
Mona Abdel-Halim - Dermatology Department, Faculty of Medicine, Cairo University (Egypt) Wrote:

Hi all. The valuable comment of Dr Juan Carlos has triggered me to look in the lymphoma area. Lymphoma is also one of the important DDx of small blue cell neoplasms. I think I came up with a nice idea, could this be Lennert's lymphoma (lymphoepithelioid TCL), in which we see clusters of epithelioid histiocytes admixed with the lymphocytes??? I love these challenging cases!!!

Submitted on 26/11/2010 16:50
________________________________________
Miah Singh - (UK) Wrote:

Difficult case - lymphoma of some sort!

Submitted on 26/11/2010 15:15
________________________________________
Carlos B - (Spain) Wrote:

I also agree with 'high endothelial cells' and therefore T cell lymphoma

Submitted on 26/11/2010 15:14
________________________________________
Juan Carlos Garcés - Hospital Oncológico / Hospital Luis Vernaza (Guayaquil Ecuador) Wrote:

Good morning everyone.. I am not sure of anything in this case.. Those long branching estructures remind me postcapillary venules with very plump endothelial cells (however they could be epithelial!) in a rich lymphocyte background. I am thinking in Lymphoma..(angioimmunoblastic? ... I dont see polymorphus infiltrate )(peripheral T cell lymphoma?). Sorry if I am very wrong..I am wainting very anxiously other opinions and the rigth answer. Great weekend to everyone in all the continentes!!

Submitted on 26/11/2010 14:12
________________________________________
Eman El-Nabarawy - Cairo University (Egypt) Wrote:

Difficult case. I thought of Merckle cell carcinoma with squamous diffrentiation.

Submitted on 26/11/2010 12:21
________________________________________
Richard Carr - Warwick (UK) Wrote:

Dear Phillip and colleagues. Posted some comments on yesterday's case. Beutiful cold day with blue skies in Warwick today - I have stopped believing in global warming. Sincerely hoping we will defeat the boks at Rugby and Aussies at criket this weekend!! What joy to be alive. Regards to all, Richard

Submitted on 26/11/2010 10:02
________________________________________
Mona Abdl-Halim - Dermatology Department, Faculty of Medicine, Cairo University (Egypt) Wrote:

Sorry for the spelling mistake, I meant to say prominent nucleoli.

Submitted on 26/11/2010 09:32
________________________________________
Mona Abdel-Halim - Dermatology Department, Faculty of Medicine, Cairo University (Egypt) Wrote:

Very difficult case. I will give it a try. Solid sheets of small atypical blue cells. Larger cells are sprinkled about the blue cells containing rounded nuclei, prominent nuclei and I am not sure but I think marginated chromatin is seen in some of them. Could this be a neuroblastoma with neuronal differentiation??? Could not identify clearly neuropoil formation, may be it is not that well differentiated. Or may be some other primitive neuroectodermal tumor. Looking forward to know what this case is!!!

Submitted on 26/11/2010 09:30
________________________________________
Phillip H. McKee - Overseas consultations (Arizona, USA) Wrote:

The case was kindly shared with me by Dr Richard Carr, from the U.K.

Submitted on 25/11/2010 23:05
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