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CASE REPORT
Year : 2020  |  Volume : 47  |  Issue : 3  |  Page : 188-191

Amyloidosis of submandibular lymph node masquerading as a salivary gland tumor: A rare case report with review of literature


1 Department of Pathology, School of Medicine, Dr. D Y Patil Deemed to be University, Navi Mumbai, Maharashtra, India
2 Department of Oral and Maxillofacial Surgery, School of Dentistry, D Y Patil Deemed to be University, Navi Mumbai, Maharashtra, India

Date of Submission08-Jun-2020
Date of Acceptance18-Jul-2020
Date of Web Publication21-Jan-2021

Correspondence Address:
Dr. S Sudhamani
Department of Pathology, School of Medicine, Dr. D Y Patil Deemed to be University, Nerul, Navi Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jss.JSS_55_20

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  Abstract 


Amyloidosis is a disease characterized by the extracellular deposition of fibrillary proteins causing organ dysfunction. Submandibular lymphadenopathy is a rare clinical presentation of amyloidosis. Majority of the patients progress to develop systemic diseases and usually have AL amyloidosis. Here, we report a case of a 40-year-old male, who presented with a left submandibular swelling for 3 years. The clinical diagnosis was malignant submandibular salivary gland tumor. Systemic examination was unremarkable. Histopathological examination of the excised swelling revealed effacement of lymph node architecture by an eosinophilic Schiff poor material. Positive Congo red stain with apple-green birefringence on polarization confirmed it as amyloidosis. Serum protein electrophoresis was negative for M Band. Immunohistochemistry revealed kappa light-chain restriction in the deposits, characterizing it as localized primary amyloidosis of the submandibular lymph nodes.

Keywords: Amyloidoma, amyloidosis, submandibular swelling


How to cite this article:
Mukharji S, Shruthi K P, Date A, Sudhamani S, Rao R. Amyloidosis of submandibular lymph node masquerading as a salivary gland tumor: A rare case report with review of literature. J Sci Soc 2020;47:188-91

How to cite this URL:
Mukharji S, Shruthi K P, Date A, Sudhamani S, Rao R. Amyloidosis of submandibular lymph node masquerading as a salivary gland tumor: A rare case report with review of literature. J Sci Soc [serial online] 2020 [cited 2021 Apr 16];47:188-91. Available from: https://www.jscisociety.com/text.asp?2020/47/3/188/307601




  Introduction Top


Amyloidosis is a disease characterized by the extracellular deposition of fibrillary proteins causing organ dysfunction. In the mid-19th century, Virchow described it as amyloidosis due to its starch-like tinctorial properties at autopsy. It can be hereditary or acquired and localized or systemic. Secondary or acquired is the most common form. Amyloid deposition can occur in any tissue and histologically give characteristic apple-green birefringence under polarized light with Congo red stain.[1] Isolated lymph node amyloidosis is relatively rare, as it is seen usually secondary to systemic amyloidosis. Mediastinal and hilar lymph nodes are commonly affected, and amyloidosis of the submandibular lymph node is generally not seen and accounts for 10' of cases.[2] Only 2' of patients with amyloidosis present with lymphadenopathy as the initial symptom. We report this case because of its rarity and unusual presentation.


  Case Report Top


A 40-year-old male presented with an enlarged, painless, left-sided submandibular swelling, gradually increasing in size for 3 years. He gave a history of cigarette smoking for 20 years. There was no history of any fever or weight loss. There was no history of any comorbidities or significant history. There was no family history of any illness. Physical examination showed a well-defined, firm, partially mobile swelling of size 7 cm × 6 cm, in the left submandibular region [Figure 1]. The lesion was nontender and was not attached to the mandibular bone or skin. There was no other lymph node enlargement or pallor. Examination of the oral cavity was normal. The remaining head and neck examination and systemic examination were unremarkable.
Figure 1: Left-sided submandibular swelling

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Computed tomography (CT) examination of the head and neck demonstrated a well-defined, oval-shaped, smooth marginated, heterogeneous mass density lesion measuring 6.2 cm × 5.8 cm × 4.6 cm involving the left submandibular space from which the left submandibular gland was not seen separately. Multiple coarse and punctate calcifications were seen scattered throughout the lesion. No obvious cortical breach of the mandible was noted. Thyroid showed normal morphology. Fine-needle aspiration was nondiagnostic due to scant cellularity. The swelling was excised under general anesthesia. Intraoperatively, the mass could not be separated from the submandibular salivary gland, and therefore a diagnosis of salivary gland tumor was considered. Frozen section was requested because of the suspicion of malignancy. On frozen section, the tissue showed acellular, eosinophilic material with many giant cells and calcified areas. There was no evidence of atypia or malignancy. Therefore, a diagnosis of benign salivary gland tumor was given.

We received a soft tissue mass measuring 9 cm × 7.5 cm × 5 cm. The salivary gland was seen attached to the mass measuring 5 cm × 3 cm × 1.5 cm. The external surface was bosselated, with an intact capsule along with fibrofatty tissue. Cut surface of the mass was gritty, yellowish, and homogenous. A calcified nodule was noted measuring 2.5 cm in diameter [Figure 2]. Eleven lymph nodes were isolated from the surrounding tissue ranging in size from 1 cm × 1 cm to 0.5 cm × 0.5 cm.
Figure 2: Gross image (cut surface)

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On microscopy, the sections studied showed a well-circumscribed mass with normal lymph node architecture at the periphery, compressed by large islands of acellular eosinophilic extracellular material [Figure 3], which was Schiff poor. The surrounding area showed chronic inflammatory cell infiltrate along with foreign body type of giant cells. Congo red staining showed a bright pink color, which under polarized light revealed apple-green birefringence [Figure 4]. This confirmed the diagnosis of lymph node amyloidosis, ruling out the possibility of salivary gland tumor. Calcified areas were also noted in the lesion. The sections from the adjacent salivary gland and lymph nodes were unremarkable. Amyloid in cervical lymph nodes may be primary/secondary or rarely from metastasis of medullary thyroid carcinoma.
Figure 3: H and E, ×40 lymph node with eosinophilic acellular amorphous deposits

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Figure 4: Congo red stain (inset) ±400 with polarized microscopy showing apple-green birefringence of amyloid deposits

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Further evaluation for ruling out systemic involvement was done by hemogram, peripheral smear, erythrocyte sedimentation rate, electrolytes, renal function test, liver function test, electrocardiogram, echocardiogram, and chest X-ray, which were all within normal limits. Serum protein electrophoresis was negative for M band. There was no hypercalcemia, renal failure, anemia, or lytic lesions in the bone on X-ray. Serum-free light-chain assay and bone marrow study were not done. Thyroid CT scan and thyroid function tests were also within normal limits.

On pretreatment of the section with potassium permanganate before Congo red staining, AA type of amyloid loses its affinity for Congo red. In our case, the amyloid deposits got stained even after pretreatment with potassium permanganate, suggesting it to be of AL type.?[3] Immunohistochemistry helps differentiate between primary and secondary amyloidosis. In this case, the immunohistochemistry for kappa light chain was intensely positive in the deposits, whereas lambda light chain was negative, confirming the diagnosis of AL type of amyloidosis. Hence, the final diagnosis of isolated primary amyloidosis of the submandibular lymph node was made. Postoperatively, the patient is asymptomatic and is on follow-up.


  Discussion Top


Amyloidosis is a chronic disorder which can be localized or systemic and have varied presentation depending on the site of amyloid protein deposition. Amyloidosis can also be idiopathic/primary or associated with multiple myeloma (AL) or secondary due to infection or inflammation (AA).[4] Localized amyloidosis involves amyloid deposition in a single organ; it can occur in tracheobronchial tree, gastrointestinal tract, breast, skin, and lymph node. Lymph node amyloidosis is a distinct entity and can present with localized and/or systemic involvement.[5] Lymph node as a primary site of amyloidosis is very rare as it is usually secondary to primary elsewhere. Fu et al.[6] evaluated amyloidosis presenting as lymphadenopathy and found that in patients with thoracic involvement and systemic amyloidosis, 75' of patients have been reported to have predominantly mediastinal or hilar lymph nodes. They studied total 3008 cases of amyloidosis and found that 53' were men with a median age of 63 years. Out of these, only 47 (2') patients were diagnosed based on lymph node biopsy.[6] Further, in their study, the common lymph nodes involved in decreasing order of frequency were mediastinal (30'), cervical (23'), inguinal (17'), axillary (15'), and others (15').[6] Out of these, only 30' of patients had lymphadenopathy as the only symptom (isolated) without any evidence of organ involvement, and 83' showed AL-type amyloidosis.[6] It is said that, even though initially lymph node enlargement may be the only presentation without systemic disease, the patient need regular follow-up as they may develop organ disease later. In the review study by Fu J et al.,[6] 71.5' of patients (10 out of 14) subsequently developed a systemic lymphoplasmacytic process over a median period of 10 months. In a study of 236 cases of amyloidosis, Kyle and Bayrd[1] found submandibular swelling as the initial presenting feature only in 10' of cases. The frequency of lymph node involvement in systemic AL amyloidosis ranges from 17' to 37'.[7] Lymph node amyloidosis is said to be associated with lymphoplasmacytic lymphoma, marginal zone lymphoma, and chronic lymphocytic leukemia. It is also argued that localized lymph node amyloidosis often shows AH component, probably due to its size and heavy chains. There is no known risk factor which predicts the chances of localized lymph node amyloidosis. Still, the absence of measurable peripheral monoclonal protein and AH type deposits shows a favorable outcome. In evaluating localized lesion like a lymph node, biopsy or excision of the lesion is the mainstay for diagnosis. Histopathological examination, with the help of Congo red stain, confirms the diagnosis. Four patterns of amyloid deposition are identified: lymph node vessel involvement, follicular deposition, diffuse deposition, and a combination of follicular and diffuse deposition.[2] In our case, a combination of follicular and diffuse deposition was observed.

Prognosis of the patients depends on whether the lesion is primary or secondary, and if it is localized or systemic. Primary, localized lesion as in our case has a good prognosis and only needs excision.[8] Systemic disease will further need chemotherapy.


  Conclusion Top


Even though rare, localized lymph node amyloidosis should be considered in the differential diagnosis of isolated lymph node swellings even in the absence of other associated systemic diseases. Patients have excellent prognosis but need regular follow-up as majority are shown to progress to systemic amyloidosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name 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.



 
  References Top

1.
Pietruszewska W, Wagrowska-Danilewicz M, Klatka J. Amyloidosis of the head and neck: a clinicopathological study of cases with long term follow-up. Arch Med Sci 2014;10:846-52.  Back to cited text no. 1
    
2.
Kyle RA, Bayrd ED. Amyloidosis: review of 238 cases. Medicine (Baltimore) 1975;54:271-99.  Back to cited text no. 2
    
3.
Gilbertson JA. Amyloid. In: Suvarna SK, editor. Bancroft's Theory and Practice of Histological Techniques,8th ed.London: Elsevier Limited;2019. p:231-53.  Back to cited text no. 3
    
4.
Clevens RA, Esclamado RM, DelGaudio JM, Myers MW. Amyloidoma of the Neck: Case report and review of literature. Head & Neck 1994;16:191-5.  Back to cited text no. 4
    
5.
Dhakal B, Harrington AM, Stadler ME, D' Souza A. Localized Lymph node Light Chain Amyloidosis. Case Rep Hematol. 2015;2015:816565. doi: 10.1155/2015/816565.   Back to cited text no. 5
    
6.
Fu J, Seldin DC, Berk JL, Sun F, O'Hara C, Cui H, et al. Lymphadenopathy as a manifestation of amyloidosis: a case series. Amyloid 2014;21:256-60.  Back to cited text no. 6
    
7.
Park JH, Kwon JH, Kim JW, Cho HJ, Kim KH, Chung DH, et al. Generalized primary amyloid lymphadenopathy. Korean J Hematol 2009;44:320-4.  Back to cited text no. 7
    
8.
Newland JR, Linke RP, Lennert K. Amyloid deposits in lymph nodes: a morphologic and immunohistochemical study. Hum Pathol 1986;17:1245-9.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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