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Alveolar Rhabdomyosarcoma with CD 56 Positivity: A Mimic of Extranodal NK/T Cell

Lymphoma

Uma Kumar, P,bhiyit Das, Rani Sahu, and Pinky Kachhap

Dear Sir,

Alveolar rhabdomyosarcoma (ARMS) is a distinct subtype of rhabdomyosarcoma. It usually arises in

extremities and axial musculature.[1] These lesions affect all ages and are more common in
adolescents and young adults, unlike other subtypes which are more common in childhood.[12] Head
and neck are uncommon sites of ARMS.[1] Alveolar rhabdomyosarcoma is extremely diffi-
cult to distinguish from other primitive round cell neoplasms without immunohistochemistry
and/or genetic study. Expression of CD 56 is seen in 30-40% of ARMS.[2] We represent an un-
usual case of alveolar rhabdomyosarcoma with CD56 positivity which was diagnosed outside as
extranodal NK/T cell lymphoma on flow-cytometry. CD56 is expressed in hematolymphoid and
nonhematolymphoid neoplasm. Hematolymphoid neoplasm includes NK/T cell lymphoma,
Myeloid sarcoma, Pediatric anaplastic large cell lymphoma, plasmacytoid dendritic cell neoplasm,
gamma delta (y(S) T cells, and activated CD8+ T cells as well as on dendritic cells (DCs). Other non-
hematolymphoid neoplasms include alveolar rhabdomyosarcoma, meningioma, pulmonary
meningothelial-like nodules, Merkel cell carcinoma, Neuroblastoma (adult), neuroendocrine carci-

nomas, pancreatic neuroendocrine tumor, pheochromocytoma, synovial sarcoma, and Wilms tu mor.
However, we could establish an accurate diagnosis on careful observation of cytomorpholog-
ical features and immunohistochemistry on cell blocks.

A 25-year-old female presented with a nasal mass with recent-onset exertional dyspnea and occa-
sional epistaxis. She was diagnosed outside with extranodal NK/T cell lymphoma on flow-cytome-
try and was referred to our hospital for further treatment. On CECT PNS there was a soft tissue mass
in the left anterior nasal cavity inseparable from the left-sided nasal turbinates, septum, and

114
 hard palate. CT guided fine needle aspiration cytology was done, as the material was too scant
only cell blocks were made. H and E sections showed moderate cellularity and sheets of dyscohe-
sive cells. These cells were small round cells having irregular hyperchromatic nuclei, inconspicu-
ous nucleoli, and scant to moderate eosinophilic cytoplasm. Few mitotic figures were also seen. On
immunohistochemistry these cells were positive for desmin, myogenin, myo D1, and CD 56. The cells
were negative for LCA and cytokeratin. A diagnosis of alveolar rhabdomyosarcoma was
rendered.

Cytologically alveolar rhabdomyosarcoma shows singly dispersed small blue round cells with oc-
casional eccentric nuclei and abundant eosinophilic cytoplasm. Differential diagnosis includes ma-
lignant lymphoma, Ewings sarcoma, neuroblastoma, retinoblastoma, nephroblastoma, synovial
sarcoma, and poorly differentiated carcinoma. However, it is difficult to make a definite diagnosis on
cytological findings alone.[2 3] On-site evaluation of these tumors on FNAC is difficult due to their
resemblance to hematological malignancies. As a result, the aspirate undergoes immunophe-
notyping to exclude non-Hodgkin lymphoma (NHL). The strong positivity of CD 56 on im-
munophenotyping can lead to an erroneous diagnosis, as seen in our case. However, pathologists
should be alerted by CD 45 negativity.

There are some differentiating cytomorphological features of NK/T cell lymphoma which differen-
tiate alveolar rhabdomyosarcoma. The Cytomorphological feature of NK/T cell lymphoma is the
hypercellular pattern with dyscohesive sheets of the polymorphous infiltration of the small,
medium, and large atypical lymphocytes interspersed with plasma cells, macrophages, neutrophils, and
occasional histiocytes. Cells show scant to moderate amounts of cytoplasm with pleomorphic nuclei
and small nucleoli. These atypical cells are positive for CD 56 and CD 45 on cell block sec-
tions, while FNAC of rhabdomyosarcoma revealed dyscohesive sheets of small blue round cells with
occasional eccentric nuclei and abundant eosinophilic cytoplasm [Figure la-b]. Few cells are arranged
in an alveolar pattern. Immunohistochemically, the tumor cells expressed CD56 but CD
-:'= negative [Figure 2a-f]. Pathologists should be alerted by CD 45 negativity.

Cellblock of fine-needle aspiration from nasal mass shows the polymorphous population of tumor cells with ec-
centrically placed hyperchromatic nuclei with abundant eosinophilic cytoplasm. Mitosis also seen. (H and E; Magnification (a) x100 & (b)
x 400)

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Win KT et al. [4] reported a similar case of ARMS where the initial diagnosis of Extranodal NK/T
cell lymphoma was made on flow-cytometry. Yasuda T et aija] also concluded in their study that
establishing the diagnosis of ARMS in older adults is usually neglected due to its rarity in this age
group, lack of a characteristic alveolar pattern, and CD 56 positivity.

To conclude, CD 56 positive cells should be cautiously studied, we should employ a panel of mark-
ers, including desmin, myogenin/MyoD1, and genetic study in the diagnosis of small round cell tu-
mors so that the possibility of this condition is not overlooked.

Key message

We should employ a panel of markers including desmin and myogenin/MyoD1 in the diagnosis of
small round cell tumors so that the possibility of this condition is not overlooked.

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 infor-
mation to be reported in the journal. The patients understand that their names 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

314
 Conflicts of interest

There are no conflicts of interest.

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