Plasma Cell Neoplasm Manifesting Initially as a Sub-Cutaneous Supra-Orbital Swelling

Abstract

Multiple myeloma is a plasma cell neoplasm seen usually in patients over 50 years of age. Some cases may be asymptomatic initially and are detected during a routine test like complete blood count. They only require a close follow-up and monitoring. However, around 1% of these monoclonal gammopathy of undetermined significance progress to multiple myeloma every year and then they need to be taken care of by chemotherapy, targeted therapy, bisphosphonates and 6 monthly urine and bone examinations. Here, we present a case of 35-year-old female with an initial symptom of a vague backache along with a left subcutaneous supra-orbital swelling which was diagnosed as multiple myeloma by aspiration cytology and confirmed by ancillary tests. She has since been on treatment with bortezomib and prednisone and is responding well.

Introduction

What was known?

Plasma cell neoplasms occur predominantly in adult males, more than 50 years of age, usually present with bone pains or abnormal proteins in urine, with an associated high risk in case of a positive family history.

Plasma cell neoplasms are diseases in which there is an increase in the number of plasma cells, many of which depict atypia under the microscope in the form of increase in size, multi nucleation, vacuolation and loss of nuclear cartwheel pattern. Plasma cell neoplasms can be benign or malignant.

There are several types of plasma cell neoplasms viz. Monoclonal Gammopathy of Undetermined Significance (MGUS), Plasmacytoma and Multiple Myeloma.

Multiple myeloma and other plasma cell neoplasms may cause a condition called amyloidosis. Age can affect the risk of plasma cell neoplasms. Blood, bone marrow, and urine examination are used to detect, diagnose and treat multiple myeloma and other plasma cell neoplasms.

Case Report

A 35-year-old female presented with off and on pain in the lower back for the last 5 months, and a left subcutaneous supra-orbital swelling for the last 3 months [Figure 1]. For lower backache, she consulted a gynecologist but was not relieved. There were no systemic complaints or significant history of tuberculosis, tingling sensations, or any previous treatment.

Figure 1.

Clinical photograph showing the left supra-orbital swelling

The swelling measured 1.5 cm × 1.0 cm in size and was firm, mobile, and nontender. There were no palpable lymph nodes or any other similar swelling anywhere in the body.

A clinical suspicion of metastatic malignancy/tuberculosis was suggested, and the patient sent to the Pathology Department for fine needle aspiration cytology (FNAC).

(FNAC) smears (Hematoxylin and Eosin, Giemsa stained) were richly cellular and revealed clusters and sheets of atypical cells lying in a clear background. Tumor cells were pleomorphic, having central to eccentric nuclei (some were multinucleated), occasionally prominent nucleoli, and cytoplasmic vacuolation [Figure 2].

Figure 2.

Fine needle aspiration cytology smear showing atypical plasma cells (H and E, ×100)

Tuberculosis was ruled out (Ziehl Neelsen negative) and the smears were subjected to immunocytochemistry for confirmation of the origin of cells. CD138 came out to be diffusely positive in the cytoplasm as well as cell membrane and cytokeratin were negative [Figure 3].

Figure 3.

Immunocytochemistry showing membranous and cytoplasmic positivity of CD138 in tumor cells

Peripheral blood smear and bone marrow smears, renal function tests were, however, unremarkable and no abnormal “M” protein was detected in 24 h-urine test.

The case at this level was discussed with the clinician in charge, and he requested for serum electrophoresis which showed elevation in gamma globulin chain by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Simultaneous 99mTc-MDP whole body scan also suggested the skeletal involvement of the frontal and vertebral bones by a malignant lesion.

The patient was put on bisphosphonates along with bortezomib (biweekly nine cycles) and responded well. She was also advised a 6 monthly follow-up for urine tests, bone evaluation and any other problem that might develop.

Discussion

Multiple myeloma is a condition of malignant plasma cell proliferation derived from a single B cell lineage. These cells produce monoclonal immunoglobulins, most commonly either IgG or IgA.[1,2]

Multiple myeloma is clinically staged between I and III based on all these findings. 1–5% of the cases, however, do not secrete the hallmark “M” protein, and they are known as “nonsecretor” myelomas.[3,4] At the time of initial diagnosis, all patients should be subjected to complete blood count, serum calcium, serum creatinine levels, and a radiographic survey of the skeleton.[2] Once the diagnosis is made, these investigations must be repeated every 6 months to check the response to treatment as well as to detect “smouldering myeloma.”[5]

The patient in our case presented with a subcutaneous swelling instead of the usual manifestations that could arise a suspicion of plasmacytoma or multiple myeloma. Whole body bone scan performed after FNAC, however, showed lytic lesions in the spine, serum protein electrophoresis showed gamma globulin peak and immunocytochemistry of the supra-orbital swelling smears confirmed the nature of the lesion.

The International Myeloma Working Group has issued updated criteria for the diagnosis of multiple myeloma which have been published in the Lancet oncology in 2014 and are briefed in the table below.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

What is new?

A female patient, age <40 years, presenting with a less common manifestation of any plasma cell neoplasm, that is, a subcutaneous swelling and multiple myeloma confirmed by cytology.