A 28 years old female presented to ENT outdoor with complaint of growth over hard palate and pain in the lesion for 2 months. Patient had episodes of bleeding from the growth off and on. The growth was polypoidal, exophytic, firm and bled on touch. The general physical examination did not reveal any abnormality. However, she also had pain in left lower limb and was lethargic most of the times.
Her laboratory reports revealed the following: Hb- 9.6 gm/dl, TLC- 13000/cmm, DLC- P78 L18 M2 E2, platelets were normal. ESR 110 mm/hr, blood urea 35 mg%, S. Creatinine- 0.7 mg%. fungal stains and Xpert TB were negative.
Computed tomography (CT Scan) showed an evidence of heterogenously enhancing nodular oral cavity mass lesion causing destruction of hard palate and alveolar process of maxilla and it was bulging into the nasal cavity (Fig. 1). Further, CT head showed multiple lytic lesions in calvaria (Fig. 2). Positron Emission Tomography (PET) revealed hypermetabolic lytic lesions involving axial and appendicular skeleton. Keeping in view her symptomatology, Magnetic Resonance Imaging (MRI) of left knee was also performed which revealed diffuse marrow infiltrative disorder possibly leukemia or lymphoma. Biopsy from hard palate growth was done. It showed a diffuse infiltration by medium sized tumor cells, of plasmablastic morphology with fine reticular nuclear chromatin, large nucleus, with very little perinuclear hof (Fig. 3). Immunohistochemistry (IHC) Panel applied revealed strong positivity of tumor cells for kappa light chains, while these cells were lambda chains negative. CD 38 and CD 138 were also positive. CD20, CD5, CD 10, CD 3, BCL 6 and Cyclin D1 were negative. Ki67 index was high (60%). EBER (ebstein barr virus encoded RNA) was also negative, ruling out the differential of plasmablastic lymphoma (Fig. 4). So, a final diagnosis of plasmablastic myeloma was made.
Serum protein electrophoresis yielded hypoalbuminemia and M spike in gamma region (2.4 g/dl), further corroborating the diagnosis of plasma cell disorder. Thus, the diagnosis of plasmablastic myeloma was confirmed. The case was discussed in multidisciplinary tumor board. She was taken up for surgery followed by chemo-radiation. But unfortunately, she did not respond well to treatment and finally succumbed to her illness after 6 months.
CT scan showing heterogenously enhancing nodular lesion causing destruction of hard palate and alveolar process of maxilla.