|Year : 2014 | Volume
| Issue : 5 | Page : 683-685
Disseminated non-Hodgkin's lymphoma presenting as unilateral parotid gland enlargement with facial nerve palsy
Maheswar Samanta, Biswajyoti Ratha, Ashok Kumar Mallik, Manasi Mishra
Department of Neurology, S.C.B. Medical College, Cuttack, Odisha, India
|Date of Web Publication||10-Sep-2014|
Department of Neurology, S.C.B. Medical College, Cuttack - 753 007, Odisha
Source of Support: None, Conflict of Interest: None
The head and neck is the most common area for the presentation of lymphoproliferative disorders. Primary involvement of salivary glands is uncommon. Non-Hodgkin's lymphoma constitutes a group of malignancies those arises from cellular components of lymphoid or extra nodal tissues. Here we report a case of a 70-year-old male patient who presented with left parotid gland enlargement with left facial nerve palsy and involvement of other nodal sites, which is rarely reported in the literature.
Keywords: Diffuse large B-cell lymphoma, lymphoma non-Hodgkin, salivary glands
|How to cite this article:|
Samanta M, Ratha B, Mallik AK, Mishra M. Disseminated non-Hodgkin's lymphoma presenting as unilateral parotid gland enlargement with facial nerve palsy. Med J DY Patil Univ 2014;7:683-5
|How to cite this URL:|
Samanta M, Ratha B, Mallik AK, Mishra M. Disseminated non-Hodgkin's lymphoma presenting as unilateral parotid gland enlargement with facial nerve palsy. Med J DY Patil Univ [serial online] 2014 [cited 2020 Jul 12];7:683-5. Available from: http://www.mjdrdypu.org/text.asp?2014/7/5/683/140500
| Introduction|| |
Many times head and neck lymphadenopathy may be caused by lymphomas.  Extra nodal lymphomas are seen almost exclusively as non-Hodgkin's lymphoma (NHL) and these constitute 10-20% of all lymphomas.  Salivary gland lymphomas are quite rare and the majority of them originate from B-cells.  Swelling of unknown origin presenting as NHL of the head and neck may prove to be a challenge for diagnosis.  This report described a case of 70-year-old male manifested as having both nodal and extra nodal involvement of unilateral Lt. Parotid gland enlargement with Lt. Facial nerve palsy.
| Case Report|| |
A 70-year-old male patient presented with rapidly progressive painless swelling of the left parotid for 2 months. The patient had a history of low grade fever or unexplained weight loss for last 2 months. He noticed deviation of mouth to right recently. There was no change in his voice.
Physical examination revealed the patient to be moderately built and poorly nourished with signs of anemia. Ocular examination was normal. The spleen and liver were not palpable. Other systemic examinations, including that of the respiratory, cardiac, and central nervous systems were normal.
Extra orally he had a diffusely enlarged, nontender, firm swelling of Lt. Parotid gland [Figure 1]a without any local signs of inflammation or fixity to surrounding structures and a rubbery consistency of the cervical lymph nodes (along with those of the sub mental, submandibular, and jugulodigastric groups), axillary, and inguinal groups. Left side lower motor neuron type seventh nerve palsy noted with deviation of mouth to right and positive Bells sign [Figure 1]b. Anterior and posterior rhinoscopy were normal. Multiple small nodules had seen in buccal mucosa bilaterally.
A routine hemogram showed normocytic anemia (hemoglobin level of 8.8 gm%) with erythrocyte sedimentation rate, total leukocyte count and platelet count within the normal limit. Tests for human immunodeficiency virus (HIV) I and II were nonreactive. Renal, Hepatic profile, and computed tomography scan brain were normal. Bone marrow aspiration cytology revealed an interstitial pattern of marrow involvement.
Chest radiograph was unremarkable. Ultrasonography studies confirmed the enlargement of the left parotid with homogenous texture, lobular contour. Main parotid duct not visualized with multiple enlarged nodes along carotico- jugular vessel and digastrics region. Ultrasonography of the abdomen shows no hepato-splenomegaly with preserved architecture.
Fine-needle aspiration cytology (FNAC) of left cervical lymph node [Figure 2]a shows infiltration of capsule and pericapsular fat. Lymph node biopsy [left cervical, [Figure 2]b] shows effacement of lymph node architecture, infiltration of capsule with monotonous population of cells with round nuclei, scanty cytoplasm clumped chromatin, indistinct nucleoli, good number of mitotic figures. FNAC from the lt. Parotid [Figure 3]a showed a monomorphic population of lymphoid cells with the absence of Reed-Sternberg cells presents discohesively having coarse chromatin, prominent nucleoli. Nuclear outline is irregular. Back ground shows few mature lymphocytes, which was suggestive of NHL. Incisional biopsy of the Lt. Parotid gland [Figure 3]b revealed effacement of parotid architecture with diffuse infiltration by sheets of monomorphic moderately large cells, with moderate cytoplasm and hyper chromatic nuclei, inconspicuous nucleoli. Mitotic figures were frequently identified.
Immunohistochemical staining shows positive staining for CD20, immunonegative for CD10, CD3, CD5, and CD23.
Diagnosis of NHL-diffuse large B-cell type was concluded based on FNAC and biopsies of the parotid glands and lymph nodes, supported by immunohistochemical positivity for CD20 marker. The patient was referred to the oncology department regarding chemotherapy and radiotherapy.
| Discussion|| |
Non-Hodgkin's lymphoma arises from a lymphocyte progenitor and comprises a heterogeneous group of highly diverse malignancies.  25-40% of NHLs are extra nodal in origin  and usually manifest in the gastrointestinal tract followed by the head and neck region.  The majority of salivary gland lymphomas (70-80%) arise in parotid gland and most are low grade NHL. NHL mostly occurs in the pediatric age group in the head and neck region;  however, it may be seen in an older age group, such as in the present case of a 70-year-old male.
The etiological factor for primary lymphoma of the salivary gland region is unclear.  Oral lymphomas are frequently seen with acquired immune deficiency syndrome. The present case was seronegative for HIV.
The most common presentation of primary oral and para oral lymphoma is a painless local mass with superficial ulceration.  In our case, patient presented with progressive painless swelling of the left parotid glands and left seventh nerve palsy in addition to nodal involvement. Based on the morphology, biopsy of parotid, lymph node (cell lineage,) and immunohistochemical findings, the present case was categorized as NHL of diffuse large B-cell type.
To determine the prognosis and to guide therapy for NHL, staging is important. The Ann Arbor staging system is one of the most widely used systems. According to the Ann Arbor system, Stage IVEB (Stage IV: Diffuse or disseminated foci of involvement of one or more extra lymphatic organs or tissues; E: Extra nodal organ involvement; B: Presence of systemic symptoms such as fever and loss of weight) was ascribed.
Non-Hodgkin's lymphoma is associated with significant morbidity. Early stages of NHL (minimal lymph node involvement) are more manageable with a greater prospect of long-term disease-free survival, whereas advanced stages (widespread in the lymph nodes) of the disease have a lower prognostic index.  A diagnostic dilemma often occurs for oral physicians when these lymphomas occur at extra nodal sites.  The present case is a disseminated NHL with left parotid enlargement and facial palsy. Though the involvements of both nodal and extra nodal sites were present, the patient complained mainly swelling of Lt. parotid gland. Since NHL constitutes only 1.7% of salivary gland malignancies,  a clinical diagnosis of lymphoma is rarely suspected at initial presentation.
| Conclusion|| |
An old patient with unilateral parotid swelling, facial nerve palsy and generalized Lymphadenopathy was found to have NHL after evaluation. This is a rare but important differential diagnosis to consider in patients with parotid swelling and lymphadenopathy because early diagnosis, staging and treatment can improve survival.
| References|| |
|1.||Revanappa MM, Sattur AP, Naikmasur VG, Thakur AR. Disseminated non-Hodgkin′s lymphoma presenting as bilateral salivary gland enlargement: A case report. Imaging Sci Dent 2013;43:59-62. |
|2.||van der Waal RI, Huijgens PC, van der Valk P, van der Waal I. Characteristics of 40 primary extranodal non-Hodgkin lymphomas of the oral cavity in perspective of the new WHO classification and the International Prognostic Index. Int J Oral Maxillofac Surg 2005;34:391-5. |
|3.||Nadendla LK, Meduri V, Paramkusam G. Imaging characteristics of diffuse large cell extra nodal non-Hodgkin′s lymphoma involving the palate and maxillary sinus: A case report. Imaging Sci Dent 2012;42:111-4. |
|4.||Newton R, Ferlay J, Beral V, Devesa SS. The epidemiology of non-Hodgkin′s lymphoma: Comparison of nodal and extra-nodal sites. Int J Cancer 1997;72:923-30. |
|5.||Roh JL, Huh J, Suh C. Primary non-Hodgkin′s lymphomas of the major salivary glands. J Surg Oncol 2008;97:35-9. |
|6.||Batsakis JG. Primary lymphomas of the major salivary glands. Ann Otol Rhinol Laryngol 1986;95:107-8. |
[Figure 1], [Figure 2], [Figure 3]