SOFT Case 3 (15th Dec 2004)

A 28 year old female had a progressive swelling of the hard palate with loosening of the incisor teeth, since 2 months. Her Routine investigations, Bone Marrow and serum electrophoresis were normal. Images presented are the patient(1), the cytology smears(2), and the HPE(5).

Presented by Dr U Bordoloi, Dr P Baruah, Dr M K Baruah, Dept. of Plastic Surgery, Assam Medical College, Dibrugarh and MDRC, Dibrugarh

Dr (Mrs) P Baruah drpriyakshi@rediffmail.com .

DISCUSSION OF SOFT CASE 3

16 pathologists have attempted the SOFT Case 3. The following are the diagnosis offered:

Plasmacytoma 4
Plasma Cell Granuloma/ Mucous Membrane Plasmacytosis 2
NHL 2
Myoepithelioma 2
Poorly Differentiated Nasopharyngeal Ca 1
Fungal infection -Histoplasmosis/ Cryptoccosis 3
Leishmaniasis 1 Rhabdomyosarcoma 1

The images of the FNAC and the subsequent HPE of the excised specimen showed sheets of plasma cells, some of which were binucleated , together with some acute inflammatory cells specially in the superficial areas . Fungal bodies could not be demonstrated in routine or special stains. With this picture our provisional diagnosis was that of an EXTRAMEDULLARY PLASMACYTOMA inspite of the age of the patient. With the help of Dr Sanjay Navani , Breach Candy Hospital , Mumbai , IHC was done to confirm the diagnosis. However, Immunohistochemical staining showed plasma cells reactive for Kappa and Lambda chains in the ratio of 1: 2.5 . (A Kappa : Lambda ratio of 1:5 or 1: 6 or more than 1:10 is suggestive of a monoclonal proliferation.) Based upon this IHC report we reviewed our diagnosis to a PLASMA CELL GRANULOMA of REACTIVE NATURE.

Plasma Cell Granulomas are completely curable by excision of the mass. But within a month after the excision biopsy was done, the patient once again presented to us, and this time with perforating ulcers in the cheek of the same side,recurrance at the same site, and the FNAB of the lesion again showed sheets of plasma cells. Thus on the basis of these developments, we once again reviewed our diagnosis , and labelled it as MUCOUS MEMBRANE PLASMACYTOSIS .

MUCOUS MEMBRANE PLASMACYTOSIS is a rare idiopathic benign disorder where the mucous membranes are infiltrated by plasma cells. The lesion has been described in nearly all orifices including the vulva, penis, buccal mucosa, palate, nasal aperture, gingiva, lips, tongue, epiglottis, and larynx. Histologically, the lesions have been described as having an acanthotic epidermis with spongiosis, and a dense submucosal cellular infiltrate consisting largely of plasma cells, which are polyclonal in nature.

These lesions are extremely difficult to treat. In spite of its benign histology, it is very progressive and in few of the reported cases even tracheostomy was required. Various modalities of treatment have been tried, but with mixed success. Corticosteroids, antibiotics, antifungal treatment, destruction of the tissue (liquid nitrogen, CO2, laser and electrocoagulation), excision of the tissue and radiation therapy, have all been tried; but no treatment clearly stands out as consistently effective.

Thanks to Drs Neha Dehiya, Ravi Shankar , Paolo Boccato , Gyanendranath M , Sachin Kale , Sangeeta Narayanan, Rashmi Patnayak , S Shaila , Atul Kapilla , Gurudutt Gupta, Usha Burra, Sonika Dahiya, Dilip Ramrakhiyani, S. Datta, Maria Mestre and Kaveri Hazarika, who have attempted the case. Dr Dilip Ramrikhiani stands out from the others in the sense that he mentioned all the differential diagnosis we had thought of.

References:
1.Mucous membrane plasmacytosis of the upper aerodigestive tract. A clinicopathologic study. Ferreiro JA, Egorshin EV , Olsen KD, Banks PM , Weiland LH . Am J Surg Pathol . 1994 Oct ; 18 (10) : 1048 -53.
2.Mucous membrane plasmacytosis : A case report and review of the literature . Rakesh Bharti, Denise R Smith. Dermatology Online Journal 9- 2003(5) : 15
3.Plasma cell granuloma of the oral cavity : a report of two cases and review of literature . Ballesteros E , Osborne BM , Matsushima AY . MOD Pathl 1998 Jan ; 11(1) : 60-4