Dural Plasmacytoma Involving Calvarium with Soft Tissue Exte
In October 2015, a 25-year-old male patient presented with gradually and progressively enlarging painless scalp swelling over the left occipital region since 6 months. On examination the patient was fully conscious and oriented to time, place, and person. There was no cranial nerve deficit, and tone was normal. Power upper limb, shoulder (abduction/adduction), elbow (flexion/extension), grip: 5/5. Lower limb, hip (abduction/adduction), knee (flexion/extension), ankle (DF/PF): 5/5, DTR++. Planter B/L flexor and no sensory system deficit was present. On investigation hemoglobin (Hb) was 13.2 g%, total leukocyte count (TLC) 10,660/mm3, differential leukocyte count (DLC) N68%L20%EO6%M06%, blood urea 22 mg%, and serum creatinine was 0.75 mg%. Viral markers were negative, and coagulation profile was within normal range.

Magnetic resonance imaging of the brain revealed large well-defined extra-axial densely but heterogeneously enhancing soft tissue mass lesion in the left parieto-occipital region, which was destroying the left parietal bone, bulging into the scalp compressing the adjacent superior sagittal sinus without obvious invasion along with mass effect and midline shift to right side. The mass measured 8 × 7.9 × 6.8 cm. No restricted diffusion or surrounding edema was seen. The findings were suggestive of a neoplastic lesion, and possibilities of atypical meningioma or sarcoma or vascular lesion were considered on radiology.

Left parieto-occipital craniotomy and gross total excision of tumor mass were done. Perioperatively tumor was pinkish, gray, highly vascular, and present extradurally involving and invading the calvarium with large extracranial extension. It was gradually dissected along the extradural plane. Dura was highly vascular. Broad dural origin was coagulated, and gross total decompression of tumor mass was done.

Biopsy received in the laboratory was large soft to firm, grayish brown, and tissue piece measuring 10 × 9 × 5 cm in size along with bony piece 3 × 2 × 1 cm. On microscopy it revealed exuberant production of amorphous, eosinophilic, fibrillary material in form of small and large nodular aggregate. Interspersed with these amorphous deposits were dyscohesive tumor cells. The tumor cells were small round to oval cells with eccentric uniform nuclei, coarse chromatin, inconspicuous nucleoli, and thin rim of granular cytoplasm and characteristic “perinuclear hof,” resembling plasma cells. This abundant deposit was congophilic, and underpolarized microscopy showed apple green birefringence.

On immunohistochemistry, the tumor cells were positive for CD138 with κ-light-chain restriction. Therefore, the diagnosis of plasmacytoma with amyloid deposition was made. Myeloma workup (serum and protein electrophoresis with immunofixation, skeletal survey, serum immunoglobulin, and free light-chain assay) was negative. The patient received radiotherapy of 46 Gy in 23 fractions to primary site. We followed up the patient to rule out progression to MM. The patient was free from systemic progression for the past 24 months although future course remains to be seen.

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