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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 70-73

Recurrent metastatic malignant granular cell tumor of the back presenting with axillary mass: A rare case report and review of the literature


Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Submission04-Oct-2020
Date of Acceptance05-May-2021
Date of Web Publication22-Dec-2021

Correspondence Address:
Kavita Mardi
Set No 14, Type VI Quarters, IAS Colony, Mehle, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijcpc.ijcpc_19_20

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  Abstract 


Malignant granular cell tumor (MGCT) is a rare aggressive tumor that comprises 0.5%–2% of all granular cell tumors. They most commonly occur on lower extremity, nuchal region, chest wall, gastrointestinal tract, and head and neck. We report fine needle aspiration and histopathological findings of a rare case of recurrent MGCT presenting with axillary mass along with a tumor on the right back in a 56-year-old female. Tumor cells with abundant granular eosinophilic cytoplasm showing pleomorphism, enlarged vesicular nuclei, prominent nucleoli, 4–5 mitotic figures/10 high-power field, periodic acid–Schiff-positive diastase-resistant cytoplasmic granules, and metastasis in the axillary lymph node were also suggestive of MGCT. Immunohistochemically, the tumor cells were positive for S-100 confirming the diagnosis.

Keywords: Fine needle aspiration cytology, granular cell tumor, malignant


How to cite this article:
Mardi K, Murgai P, Mehta P, Sarohi M. Recurrent metastatic malignant granular cell tumor of the back presenting with axillary mass: A rare case report and review of the literature. Int J Clinicopathol Correl 2021;5:70-3

How to cite this URL:
Mardi K, Murgai P, Mehta P, Sarohi M. Recurrent metastatic malignant granular cell tumor of the back presenting with axillary mass: A rare case report and review of the literature. Int J Clinicopathol Correl [serial online] 2021 [cited 2022 Jan 19];5:70-3. Available from: https://www.ijcpc.org/text.asp?2021/5/2/70/333395




  Introduction Top


The malignant granular cell tumors (MGCTs) are extremely rare, representing less than 0.5%–2% of all GCTs.[1] Both benign GCT and MGCT have been found in a wide variety of locations, including lung, heart, pelvis, bladder, vulva, abdominal wall, and esophagus.[2],[3],[4],[5],[6],[7],[8],[9],[10],[11] They occur most commonly on lower extremities. The diagnostic histological criteria have been proposed by Fanburg-Smith et al.[12] although these criteria for malignancy are still debated among pathologists. We present cytological and histopathological findings of a rare case of MGCT presenting as recurrent axillary mass in a 56-year-old female.


  Case Report Top


The patient noted the swelling for the first time in the right axillary region, 3 years back. Excision of the swelling was done, but the patient did not follow up and did not collect the report. The swelling recurred in the right axillary region after 3 years along with a mass below the inferior angle of the right scapula. The right axillary mass was of size 3 × cm 2 × cm 1 cm, nontender, firm, mobile. A similar nontender, firm mass of size 1 cm × 1 cm was also noted below the right inferior angle of the scapula. Computed tomography of the thorax and abdomen with contrast showed enhancing enlarged nodes in the right axilla measuring 4 cm × 1.8 cm with few small subcentimeter-sized nodules in both lung fields. Positron emission tomography scan revealed a primary tumor in the right inferior scapular region with metastasis in the axillary lymph nodes and bilateral lungs. Fine needle aspiration (FNA) of the axillary mass revealed clusters of tumor cells revealing pleomorphism, enlarged, irregular nuclei with fine nuclear chromatin, variably prominent nucleoli, and abundant granular basophilic cytoplasm in a lymphoid background [Figure 1]. These cytological findings were suggestive of metastatic deposits from an MGCT. Right axillary mass as well as mass over the right side of upper back was excised and sent to us for histopathological examination.
Figure 1: Fine needle aspiration smears of axillary mass revealing clusters of pleomorphic tumor cells with abundant granular basophilic cytoplasm (Giemsa, ×400)

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On microscopic examination of the axillary mass, there were nests and sheets of tumor cells diffusely relaxing the lymph node parenchyma with focal residual lymphoid follicles. Similar tumor was found in scapular mass also. The tumor cells in both sites were revealing large polyhedral to spindled tumor cells with moderate pleomorphism, irregular vesicular nuclei, prominent one to multiple nucleoli, occasional intranuclear cytoplasmic inclusions, and moderate-to-abundant granular eosinophilic cytoplasm and 4–5 mitotic figures/10 high-power field (HPF) [Figure 2]. Periodic acid–Schiff (PAS) stain with diastase revealed PAS-positive diastase-resistant granules in the cytoplasm of tumor cells [Figure 3]. Immunohistochemistry (IHC) revealed S-100 positivity in the tumor cells [Figure 4]. The case was diagnosed as MGCT of the back with metastatic deposits in the axillary lymph nodes.
Figure 2: Sheets of pleomorphic tumor cells with abundant granular eosinophilic cytoplasm (H and E, ×400)

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Figure 3: Tumor cells showing periodic acid–Schiff-positive diastaseresistant granules in the cytoplasm (PAS, ×400)

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Figure 4: Tumor cells showing positivity for S-100 (IHC, ×400)

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  Discussion Top


MGCTs are extremely rare neural tumor which was first reported by Ravich et al. in 1945. Since then, less than 100 cases have been reported so far. Among the reported cases, they are most commonly seen in lower extremities, nuchal region, chest wall, gastrointestinal tract, and head and neck. However, isolated cases have been reported in various sites, including lung, heart, pelvis, bladder, vulva, abdominal wall, and esophagus.[2],[3],[4],[5],[6],[7],[8],[9],[10]

The origin of GCTs is uncertain. Initially, it was thought that they arise from skeletal muscle, fibroblastic, histiocytic, or undifferentiated mesenchymal cell origin. More recently, based on the evidence that monoclonal antibody KP-1, which recognizes the lysosome-associated glycoprotein, CD68, reacts positively with schwannomas and GCTs, it is believed that these tumors arise from Schwann's cells. Furthermore, GCTs cytoplasmically stain for S-100 protein are closely associated with nerves and are often present in distal nerve trunks. All these features support Schwann's cell origin.

MGCT differs from benign counterpart by longer clinical duration with sudden rapid growth and larger size on presentation (median size of 4–5.0 cm, as compared to benign tumors, which in most cases are <3 cm) with frequent history of local recurrence. These tumors more commonly occur in lower limbs, unlike benign GCTs, which commonly occur in the head, neck, and tongue. MGCT-like benign tumors show female predominance (70%), with presentation usually in the fifth decade of life.

Most cases present as a large mass varying in size from 4 to 20 cm, slightly fleshy, firm, yellowish to tan brownish in color with foci of hemorrhage and degeneration. Microscopically, tumor shows sheets of polygonal cells with round hyperchromatic nuclei and abundant PAS-positive diastase-resistant granules in the cytoplasm. The tumor cells display features of malignancy.

Histological criteria of malignancy were first proposed by Fanburg-Smith et al.[12] They proposed six histologic criteria for the diagnosis of atypical GCTs and MGCTs, including necrosis, spindling, vesicular nuclei with large nucleoli, increased mitotic activity (>2 mitoses/10 HPF at ×200), high nuclear-to-cytoplasmic ratio, and pleomorphism. Neoplasms that met three or more of these criteria were classified as histologically malignant; those that met one or two criteria were classified as atypical; and those that displayed only focal pleomorphism but fulfilled none of the other criteria were classified as benign.

Nasser–Ahmed–Kowalski[13] criteria refined the above criteria and proposed that necrosis and >2 mitoses/10 HPF are the most important criteria and score 0 indicates benign granular tumor and score ≥1 indicates GCT of uncertain malignant potential. They proposed that metastasis is the only criterion to diagnose MGCT. Our case showed spindling, pleomorphism, high nuclear-to-cytoplasmic ratio, vesicular nuclei with large nucleoli, 4–5 mitotic figures/10 HPF including atypical mitotic figures, as well as metastatic deposits in the axillary lymph nodes. Thus, our case meets all the criteria to be classified as MGCT.

Diagnosis and prognostication from preoperative FNA cytology are hampered by the fact that only a few case reports on cytological features of MGCT have been published.[14] The FNA of metastatic GCT may lack cytological features of malignancy. The diagnosis necessitates clinical correlation and an understanding of the spectrum of histopathological changes in GCT and MGCT.

IHC tumor cells are positive for S-100, Ki-67, and p53 and negative for ER, PR, Her2neu, GCDFP, and PanCK. Electron micrograph shows intracytoplasmic, dense, membrane-bound lysosomes typical of GCT. Features are not helpful in distinguishing between benign and malignant lesions.

Wide local excision with regional lymph node dissection remains the main modality of treatment. However, the role of systemic chemotherapy is still debated. Ordóñez[15] reviewed 41 cases of MGCT in the literature, most of which were treated with wide local excision and found a 59% recurrence rate.[15] It is advisable to follow up these patients annually to rule out late recurrences. Radiation and chemotherapy are not advised because of the tumor's high degree of resistance.[16] Tumors typically spread via lymphatic and hematogenous routes to the lungs, liver, lymph nodes, and bones. Prognosis is poor in patients with MGCT, with frequent metastasis (>50% overall) and 30%–50% mortality over 3 years in two case series.[17]

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 initial s 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.
Elkousy H, Harrelson J, Dodd L, Martinez S, Scully S. Granular cell tumors of the extremities. Clin Orthop Relat Res 2000;380:191-8.  Back to cited text no. 1
    
2.
Jiang M, Anderson T, Nwogu C, Tan D. Pulmonary malignant granular cell tumor. World J Surg Oncol 2003;1:22.  Back to cited text no. 2
    
3.
Gualis J, Carrascal Y, de la Fuente L, Echevarría JR. Heart transplantation treatment for a malignant cardiac granular cell tumor: 33 months of survival. Interact Cardiovasc Thorac Surg 2007;6:679-81.  Back to cited text no. 3
    
4.
Berg JC, Tewari KS, Del Rosario R, Berman ML. Unusual presentation of a malignant granular cell tumor of the pelvis: Case report and literature review. Gynecol Oncol 2003;90:224-30.  Back to cited text no. 4
    
5.
Ravich A, Stout AP, Ravich RA. Malignant granular cell myoblastoma involving the urinary bladder. Ann Surg 1945;121:361-72.  Back to cited text no. 5
    
6.
Schmidt O, Fleckenstein GH, Gunawan B, Füzesi L, Emons G. Recurrence and rapid metastasis formation of a granular cell tumor of the vulva. Eur J Obstet Gynecol Reprod Biol 2003;106:219-21.  Back to cited text no. 6
    
7.
An JS, Han SH, Hwang SB, Lee JH, Min BW, Um JW, et al. Granular cell tumors of the abdominal wall. Yonsei Med J 2007;48:727-30.  Back to cited text no. 7
    
8.
Panunzi A, D'Orazi V, Toni F, Coppola GA, D'Alessandro V, Pontone S, et al. Unexpected granular cell tumor in abdominal wall: Case report and literature review. Tumori 2012;98:e18-21.  Back to cited text no. 8
    
9.
Chaudhry A, Griffiths EA, Shah N, Ravi S. Surgical excision of an abdominal wall granular cell tumour with Permacol® mesh reconstruction: A case report. Int Semin Surg Oncol 2008;5:4.  Back to cited text no. 9
    
10.
Yoshizawa A, Ota H, Sakaguchi N, Kanai S, Nakayama J, Matsuzawa K, et al. Malignant granular cell tumor of the esophagus. Virchows Arch 2004;444:304-6.  Back to cited text no. 10
    
11.
McGuire LS, Yakoub D, Möller MG, Rosenberg A, Livingstone A. Malignant granular cell tumor of the back: A case report and review of the literature. Case Rep Med 2014;2014:794648.  Back to cited text no. 11
    
12.
Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: Diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 1998;22:779-94.  Back to cited text no. 12
    
13.
Nasser H, Ahmed Y, Szpunar SM, Kowalski PJ. Malignant granular cell tumor: A look into the diagnostic criteria. Pathol Res Pract 2011;207:164-8.  Back to cited text no. 13
    
14.
Mir F, Alnajar H, Rohra P, Naumaan A, Cheng L, Gattuso P. Metastatic granular cell tumor to the breast diagnosed by fine needle aspiration cytology: A case report with review of the literature. Diagn Cytopathol 2019;47:226-9.  Back to cited text no. 14
    
15.
Ordóñez NG. Granular cell tumor: A review and update. Adv Anat Pathol 1999;6:186-203.  Back to cited text no. 15
    
16.
Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: A clinicopathologic study of 110 patients. J Surg Oncol 1980;13:301-16.  Back to cited text no. 16
    
17.
Thunold S, Von Eyben FE, Maehle B. Malignant granular cell tumor of the neck: Immunohistochemical and ultrastructural studies of a case. Histopathology 1989;14:655-7.  Back to cited text no. 17
    


    Figures

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



 

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