Cutaneous presentation on the eyelid of primary, systemic, CD30+, anaplastic lymphoma kinase (ALK)-negative, anaplastic large-cell lymphoma (ALCL).
OBJECTIVE
To report an unusual case of cutaneous presentation on the eyelid of systemic (or nodal), CD30+, anaplastic large-cell lymphoma (ALCL).


METHODS
A 39-year-old man presented with a rapidly growing exophytic mass on the left upper eyelid, with a protuberant, ulcerated aspect and with discharge. The patient showed lymph node involvement 3 months after the appearance of the lesion on the eyelid (the lesion itself appeared 1 week before examination).


RESULTS
The histopathologic and immunohistochemical diagnosis was ALCL, T-cell phenotype, strongly positive for CD43 and CD30, and negative for CD3, anaplastic lymphoma kinase (ALK), and B-cell antigens. Treatment was by radiotherapy and, later, chemotherapy (cyclophosphamide, adriamycin, vincristine, and prednisolone, CHOP) for skin recurrences and lymphadenopathies over 5 years. There has been no recurrence for more than 6 years.


CONCLUSIONS
Primary, systemic, CD30+, ALK-negative, ALCL presentations generally have a poor prognosis and tend to occur in older individuals, although the clinical outcome is highly variable and difficult to predict in individual cases. Only three cases of ALCL have been described in the ocular adnexae and none was ALK-negative.
Mencía-Gutiérrez
Enrique
E
Department of Ophthalmology, 12 de Octubre Hospital, Complutense University, Madrid, Spain. emencia.hdoc@salud.madrid.org
Gutiérrez-Díaz
Esperanza
E
Salamanca
Javier
J
Martínez-González
Miguel A
MA
D000328:Adult; D000077548:Anaplastic Lymphoma Kinase; D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D005142:Eyelid Neoplasms / Q000188:drug therapy / Q000201:enzymology / Q000276:immunology / Q000473:pathology*; D006801:Humans; D017730:Ki-1 Antigen / Q000032:analysis; D008207:Lymphatic Metastasis; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000201:enzymology / Q000276:immunology / Q000473:pathology*; D008297:Male; D011505:Protein-Tyrosine Kinases / Q000032:analysis*; D020794:Receptor Protein-Tyrosine Kinases; D016896:Treatment Outcome
D015703:Antigens, CD; D017730:Ki-1 Antigen; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases