Angiocentric T-cell lymphoma presenting as lethal midline granuloma.
BACKGROUND
Lethal midline granuloma (LMG) is a rare condition characterized by rapidly progressive midfacial destruction. Most LMG cases are angiocentric T-cell lymphomas and an association with Epstein-Barr virus (EBV) has been reported. Cutaneous involvement is poorly described and the prognosis not well documented.


METHODS
We report a case of angiocentric T-cell lymphoma of the palate that presented as LMG with concurrent discrete skin lesions composed of two distinct morphologic appearances: indurated nodules and annular plaques. The English language literature for LMG-type angiocentric T-cell lymphoma is reviewed and a survival analysis of 58 cases with follow-up data (including our own case) is performed.


RESULTS
The 1- and 5-year survival rates were 45% +/- 7% and 22% +/- 9%, respectively. Poor survival was associated with advanced age and stage. EBV DNA was detected in 16 out of 21 reported cases in which it was sought (including our case).


CONCLUSIONS
We present photographic documentation of a broader spectrum of cutaneous lesions in the LMG-type angiocentric T-cell lymphoma than has previously been described, and have confirmed the association with EBV. The prognosis is poor. Aggressive therapy such as bone marrow transplantation should be considered early in the course.
Lee
P Y
PY
Dermatoepidemiology Unit, VA Medical Center, Providence, RI 02908-4799, USA.
Freeman
N J
NJ
Khorsand
J
J
Weinstock
M A
MA
D000367:Age Factors; D016026:Bone Marrow Transplantation; D004279:DNA, Viral / Q000032:analysis; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006103:Granuloma, Lethal Midline / Q000473:pathology*; D006566:Herpesviridae Infections / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000473:pathology* / Q000628:therapy; D008297:Male; D008439:Maxillary Diseases / Q000473:pathology; D008875:Middle Aged; D009367:Neoplasm Staging; D010157:Palatal Neoplasms / Q000473:pathology* / Q000628:therapy; D010159:Palate / Q000473:pathology*; D011379:Prognosis; D012878:Skin Neoplasms / Q000473:pathology; D016019:Survival Analysis; D015996:Survival Rate; D014412:Tumor Virus Infections / Q000473:pathology
D004279:DNA, Viral