ALKINEGATIVE'ANAPLASTIC'LARGE'TICELL'LYMPHOMA'PRESENTING'AS'AN'
POSTER'21'
OSTEOLYTIC'VERTEBRAL'LESION'
'
Parnaz!Daneshpajouhnejad!and!Gabriel!Caponetti!
!
Hospital!of!the!University!of!Pennsylvania,!Philadelphia,!PA!
!
Background:!Anaplastic!large!cell!lymphoma!(ALCL)!is!a!relatively!rare!type!of!lymphoma!that!
involves!lymph!nodes!and!extraJnodal!tissues.!ExtraJnodal!sites!are!less!commonly!involved!
in!ALKJnegative!ALCL!than!in!ALKJpositive!ALCL.!Bone!involvement!by!ALCL!is!very!rare,!and!
the! differential! diagnosis! in! these! cases! most! commonly! includes! Langerhans! cell!
histiocytosis,! osteomyelitis,! and! Ewing! sarcoma! in! young! patients,! and! metastatic!
malignancy! in! older! patients.! Herein,! we! present! an! unusual! case! of! ALKJnegative! ALCL!
presenting!as!an!osteolytic!lesion.!
Case!Report:!A!72JyearJold!male!presented!with!back!pain!which!developed!7!days!prior!to!
presentation,!with!no!constitutional!symptoms.!His!physical!examination!was!unremarkable!
except! for! tenderness! to! palpation! in! the! lower! back.! Laboratory! studies! revealed! mild!
normocytic!normochromic!anemia,!thrombocytosis,!increased!alkaline!phosphatase!(ALKJP)!
and!lactate!dehydrogenase,!and!mild!hypocalcemia.!A!CT!scan!of!the!lumbar!spine!showed!a!
4.4´2.8!cm!osteolytic!mass!with!irregular!margins!and!soft!tissue!component!involving!the!L1!
vertebral! body! that! was! suspicious! for! malignancy! (Fig! 1).! H&EJstained! sections! of! a! CTJ
guided! biopsy! of! the! lesion! showed! a! diffuse! infiltrate! of! atypical! mediumJsized/large!
lymphoid! cells! with! irregular! nuclei! and! occasional! “hallmark”! morphology! (Fig! 2J4).! By!
immunohistochemistry! (Fig! 5J14),! the! atypical! lymphoid! cells! were! positive! for! CD30,! CD2,!
CD4(partial),! CD25,! perforin,! granzyme! B(partial),! TIA1(partial),BCL2,! CD43,! EMA(partial),!
and!Ki67(60%),!and!negative!for!CD45,!ALK,!CD3,CD8,CD5,CD7,CD20,PAX5,!panJcytokeratin,!
SOX10,! CD138,! and! EBER(ISH).! A! diagnosis! of! ALKJnegative! ALCL! was! rendered.! FISH! for!
DUSP22JIRF4! and! TP63! rearrangements! were! negative.! Massive! parallel! sequencing!
identified!a!TP53!diseaseJassociated!variant!(variant!allele!fraction:!88%).!A!staging!PETJCT!
demonstrated!other!bone!lesions!and!FDGJavid!pulmonary!nodules!with!features!concerning!
for! lymphoma.! With! a! stage! IVA,! an! IPI! score! of! 4! (highJintermediate! risk),! and! a! CNSJIPI!
score!of!4!(high!risk),!the!patient!was!started!on!BV+CHP!(brentuximab,!cyclophosphamide,!
doxorubicin,! and! prednisone)! and! highJdose! methotrexate! for! CNS! prophylaxis.! Following!
the!start!of!treatment,!his!back!pain!improved!and!his!platelet!counts!and!ALKJP!normalized.!
Due! to! his! highJrisk! IPI! score,! he! is! being! considered! for! consolidation! with! an! autologous!
stem! cell! transplant! in! 1st! remission.! Consolidative! radiation! to! his! vertebral! lesion! is! also!
being!considered.!
Conclusions:!This!is!an!unusual!case!of!ALKJnegative!ALCL!that!is!presented!as!an!osteolytic!
vertebral!lesion,!without!any!constitutional!symptoms,!organomegaly,!lymphadenopathy,!or!
hypercalcemia.! ALCLs! with! primary! bone! presentation! display! characteristic! clinical! and!
phenotypic! features,! and! should! be! considered! in! the! differential! diagnosis! of! osteolytic!
lesions.!