Myeloid sarcoma presenting as nasopharyngeal mass: A rare clinicopathological scenario

Himanshi Diwan; Sunil Pasricha; Ankush Jajodia; Gurudutt Gupta; Mudit Agarwal; Anurag Mehta

doi:10.4103/ijpm.ijpm_791_21

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CASE REPORT

Year : 2023 | Volume : 66 | Issue : 1 | Page : 162-164

Myeloid sarcoma presenting as nasopharyngeal mass: A rare clinicopathological scenario

Himanshi Diwan¹, Sunil Pasricha¹, Ankush Jajodia², Gurudutt Gupta³, Mudit Agarwal⁴, Anurag Mehta⁵
¹ Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
² Department of Radiology, Molecular and Transfusion Services, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
³ Department of Histopathology, Molecular and Transfusion Services, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
⁴ Department of Head and Neck Oncology, Molecular and Transfusion Services, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
⁵ Department of Laboratory, Molecular and Transfusion Services, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India

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Date of Submission	05-Aug-2021
Date of Decision	18-Dec-2021
Date of Acceptance	23-Dec-2021
Date of Web Publication	18-Jan-2023

Abstract

Myeloid sarcoma (MS) is considered as an extramedullary manifestation of acute myeloid leukemia (AML) with or without concurrent AML. It can present at any age and any site, however, nasopharynx being an extremely rare site of manifestation. MS may precede AML by weeks, months or years, thereby necessitating an early diagnosis and timely intervention and treatment. We report a case of MS in a young female who presented with nasal obstruction and epistaxis for 3 months. The present case also highlights the significance of judicious use of immunohistochemistry panel while dealing with a hematolymphoid neoplasm devoid of expression of B-cell or T cell specific markers in head and neck region.

Keywords: Hematolymphoid malignancy, myeloid sarcoma, nasopharynx

How to cite this article:
Diwan H, Pasricha S, Jajodia A, Gupta G, Agarwal M, Mehta A. Myeloid sarcoma presenting as nasopharyngeal mass: A rare clinicopathological scenario. Indian J Pathol Microbiol 2023;66:162-4

How to cite this URL:
Diwan H, Pasricha S, Jajodia A, Gupta G, Agarwal M, Mehta A. Myeloid sarcoma presenting as nasopharyngeal mass: A rare clinicopathological scenario. Indian J Pathol Microbiol [serial online] 2023 [cited 2023 Mar 20];66:162-4. Available from: https://www.ijpmonline.org/text.asp?2023/66/1/162/367987

Introduction

Myeloid sarcoma (MS) is a rare extramedullary tumor consisting of myeloid blasts with or without maturation with complete effacement of tissue architecture. The rarity of the disease can be emphasized by the fact that only limited comprehensive studies are available in the literature forbye case reports. It may occur at any age and any site albeit the not infrequent sites being skin, lymph node, gastrointestinal tract, bone, soft tissue, and testis. Rarely these tumors have been reported in head and neck region like: maxilla, soft palate, paranasal sinus, scalp, salivary gland; however, primary nasopharyngeal involvement is extremely rare with only a handful of cases described in the literature.^{[1],[2],[3],[4],[5],[6],[7]} We describe a case of MS manifesting as symptomatic nasopharyngeal mass.

Case Report

A 31-year-old female, with no significant past or family history, presented with off and on, nasal bleeding and nasal obstruction for 3 months. The routine lab investigations including complete hemogram were unremarkable (hemoglobin: 11.5 g/dL, total leucocyte count: 6200/mm³, and platelet count: 2,14,000/mm³). Magnetic resonance imaging of head and neck revealed a heterogeneously enhancing mass in nasopharynx with complete obliteration of nasopharyngeal airway, left parapharyngeal space, and pharyngeal mucosal space. The mass measured 9.5 × 5.5 × 5.2 cm with mass effect on bilateral maxillary sinuses. The mass extended into cribriform plate, sphenoid sinus, left orbit, and left lamina papyracea without any bone destruction. Multiple enlarged subcentimetric cervical lymph nodes were evident [Figure 1]. The clinicoradiological differential diagnosis was an epithelial malignancy followed by rhabdomyosarcoma (RMS). Imprint smears were not available as the biopsy was received in formalin. The biopsy from nasopharyngeal mass revealed ulcerated lining epithelium with underlying stroma infiltrated by a neoplasm consisting of small round cells arranged in sheets and cords with perivascular accentuation. Few residual minor salivary glands (mucinous) were seen. The tumor cells had scant cytoplasm, round nucleus, and inconspicuous nucleoli and showed extensive crushing artefact [Figure 2]. The histomorphological differential diagnosis considered were non-Hodgkin's lymphoma (NHL), RMS, and Ewing's sarcoma. On primary immunohistochemistry (IHC) panel, the tumor cells were immunopositive for Leucocyte common antigen (LCA), while negative for CK, Nuclear protein in testis (NUT), NKX2.2, MyoD1, and Myogenin. Subsequently, B-cell, T-cell markers, and CD30 were negative. Subsequently, both myeloperoxidase (MPO) and CD117 were positive [Figure 3]. Ki67 was found to be 80%. No kappa and lambda restriction was observed. A diagnosis of MS was rendered and a bone marrow examination was suggested, but unfortunately the patient was lost to follow-up.

Figure 1: (a-d): Coronal MR imaging shows a lobulated lesion in the sinonasal cavity without any intracranial extension. (e): The same lesion in axial reformat post-contrast MR imaging and reveals heterogeneous enhancement. (f): Coronal reformat of paranasal sinus with no bony destruction

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Figure 2: (a): Ulcerated mucosa with underlying stroma infiltrated by malignant round cell tumor infiltrating salivary glands (H and E, 100×). (b): High-power view demonstrating diffuse sheets of small round tumor cells with perivascular accentuation and extensive crushing (H and E, 400×)

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Figure 3: (a): CK highlights residual minor salivary glands (arrow) while negative in tumor cells. (b): Diffuse positivity of LCA in tumor cells. (c): Tumor cells exhibiting moderate CD117 positivity. (d): Tumor cells showing diffuse and strong MPO positivity

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Discussion

MS was historically first described by Burns as chloroma due to its green tint in 1881 and subsequently also known as granulocytic sarcoma as proposed by Rappaport. It may occur de-novo (primary) or present as relapse in a diagnosed case of AML. In some cases, it may indeed be the first manifestation of systemic disease. The most common sites of involvement are lymph nodes, skin, soft tissue, and bone, while nasopharynx is extremely rare with only few case reports of primary nasopharyngeal involvement described in the literature.^{[1],[2],[3],[4],[5],[6],[7]} Zhou et al.^[9] reviewed 17 cases of myeloid sarcoma in head and neck region and most cases involved oral cavity and none had a nasopharyngeal involvement. Although MS is well-described entity in the literature, a primary clinical presentation as a nasopharyngeal mass has been very rarely described with only a handful of cases in the literature, which is diagnostically challenging.^{[1],[2],[3],[4],[5],[6],[7],[9]}

The MS, from histomorphology point of view, are considered a malignant round cell tumor (MRCT) and needs to be differentiated from its other potential mimickers like NHL, RMS, undifferentiated nasopharyngeal carcinoma, NUT midline carcinoma, and Ewing's sarcoma. Imprint smears are often beneficial as they provide information about the adequacy of the tumor tissue. The cytomorphology of the blasts is also best assessed on Wright/Giemsa stain on imprint smears. MPO stain can be performed on imprint smears to assess the lineage of the blasts. In view of normal complete hemogram, as in the presented case, the possibility of MS was often not considered as a primary differential, especially in view of nasopharyngeal site. Therefore, an algorithm-based judicious panel needs to be applied to determine the lineage of the MRCT. MS must be considered as a potential differential when the MRCT is LCA positive while negative for B cell and T-cell markers. The other differentials included in this category are plasmacytoma, ALK-negative anaplastic large cell lymphoma (ALCL) with T-cell markers down regulation or small cell variant of ALCL. CD117, MPO, and CD163 should be applied for confirming the diagnosis of MS of myeloid origin.

MS can manifest de-novo (primary) or relapse in a diagnosed case of AML. Isolated MS as defined by the absence of an underlying acute myeloid leukemia (AML) or other myeloid neoplasm is described in a handful of case reports.

Literature suggest even isolated MS, subsequently develops bone marrow infiltration in nearly all cases within few months with a mean interval of 10 months and hence diagnosing MS is tantamount to the diagnosis of AML.^[10] These patients are therefore immediately administered combination chemotherapy of AML followed by local resection and radiotherapy. Patients treated with high-dose chemotherapy and hematopoietic stem cell transplantation have a higher probability of remission and overall survival.

Most patients of isolated MS have a better prognosis than those with marrow involvement or MS following AML as observed in several studies. Hence, an accurate and timely diagnosis and early therapeutic intervention can improve the prognosis.

To conclude, the presented case highlights the rare case of MS presenting clinically as nasopharyngeal mass. MS should be considered as a differential diagnosis in MRCT, whenever dealing with LCA positive hematolymphoid malignancy with negative B-cell and T-cell markers. A diligent histopathological assessment and judicious IHC panel is imperative to clinch the diagnosis due to its distinct therapeutic and prognostic implications.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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