Successful surgical resection of solitary plasmacytoma of the liver mimicking hepatocellular carcinoma. A case report.

Abstract:

:Solitary extramedullary plasmacitomas (SEMP) of the liver are very rare. We report the case of an elderly woman with a huge symptomatic SEMP of the liver mimicking hepatocellular carcinoma (HCC). The patient was a 89-year-old woman who presented with severe abdominal pain and a huge solid mass in the right hypochondrium. The laboratory data on admission revealed normal liver function tests. A multiphasic computed tomography (CT) showed a huge solid mass of the left hemiliver, hypoattenuating on noncontrast images, dishomogeneously hyperenhancing in the late arterial phase, with washout in the portal venous and equilibrium phases. A 18F-FDG positron emission tomography (18F-FDG PET)-CT scan documented a marked FDG uptake within the lesion, without evidence of extrahepatic metastases. We considered the clinical and radiologic findings consistent with the diagnosis of high-grade HCC with areas of intratumoral necrosis preluding to possible tumour rupture. Surgical resection was ultimately considered feasible with a reasonable risk and the patient underwent left hepatectomy with diaphragmatic resection. Pathological examination exhibited an extramedullary plasmacytoma. At immunohistochemical analysis neoplastic cells were positive for CD45, CD38, IRF4, HTPD52, kappa-chain, but negative for lambda- chain; Mib-1 proliferation index was 50%. Subsequent clinical evaluation excluded any sign of multiple myeloma, so that a diagnosis of truly localized SEMP of the liver was finally established. To our knowledge, this is the first case of a solitary extramedullary plasmacitoma of the liver undergoing successful radical liver resection. The patient is alive and well 5 years after surgery without evidence of local recurrence and of systemic disease. KEY WORDS:Extramedullary plasmacytoma, Hepatocellular carcinoma, Liver, Liver resection, Multiple myeloma. :Il plasmocitoma extramidollare solitario (SEMP) del fegato è una neoplasia estremamente rara. Viene riferito il caso di un’anziana donna affetta da un voluminoso SEMP con caratteristiche simili a quelle di un carcinoma epatocellulare (HCC). Si tratta di una donna di 89 anni giunta alla nostra osservazione con un dolore addominale piuttosto severo, di recente insorgenza, ed una voluminosa massa situata nell’ipocondrio destro. I dati di laboratorio all’ingresso non evidenziavano alterazioni della funzionalità epatica; i valori di alfa-fetoproteinemia erano nella norma; non erano presenti markers di un’infezione da virus dell’epatite B e C. Una tomografia assiale computerizzata (TC) multifasica documentava la presenza di una voluminosa neoplasia dell’emifegato sinistro, ipodensa alle immagini senza mezzo di contrasto, disomogeneamente iperdensa in fase arteriosa, e con “washout” nelle fasi portale e di equilibrio. Una tomografia ad emissione di positroni con 18F-FDG (18FFDG PET)-TC evidenziava una marcata captazione del radiocomposto da parte della neoplasia, in assenza peraltro di metastasi a distanza. Le caratteristiche cliniche e radiologiche della lesione erano interpretate come compatibili con una diagnosi di HCC scarsamente differenziato con aree di necrosi intralesionale verosimilmente in evoluzione verso la rottura spontanea. La valutazione clinica complessiva ci faceva reputare accettabile il rischio chirurgico e la paziente veniva sottoposta ad un’epatectomia sinistra con resezione parziale dell’emidiaframma destro. Il decorso postoperatorio era sostanzialmente regolare e la paziente veniva dimessa in 12° giornata postoperatoria. L’esame istologico definitivo deponeva per un plasmocitoma extramidollare. L’analisi immunoistochimica dimostrava che le cellule tumorali esprimevano CD45, CD38, IRF4, HTPD52, catene leggere kappa ma non catene leggere lambda; l’indice di proliferazione Mib-1 era del 50%. La successiva valutazione clinica permetteva di escludere qualsiasi manifestazione di mieloma multiplo; si giungeva pertanto alla diagnosi di plasmocitoma extramidollare localizzato esclusivamente al fegato. Stando ai dati della letteratura scientifica, si tratta del primo caso di SEMP del fegato sottoposto con successo ad una resezione epatica curativa. La paziente è vivente ed in buone condizioni cliniche a distanza di 5 anni dall’intervento chirurgico, in assenza di recidive locoregionali e di manifestazioni di mieloma multiplo.

journal_name

Ann Ital Chir

authors

Mirarchi M,De Raffele E,Bacci F,Cuicchi D,Lecce F,Cola B

subject

Has Abstract

pub_date

2016-01-01 00:00:00

pages

343-349

eissn

0003-469X

issn

2239-253X

pii

S0003469X16025641

journal_volume

87

pub_type

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