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Year : 2019  |  Volume : 62  |  Issue : 2  |  Page : 348-350
Superficial acral fibromyxoma: A cytohistopathological correlation in a recurrent, nonperiungual, acral lesion

Department of Pathology, Deen Dayal Upadhyay Hospital (Government of NCT), New Delhi, India

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Date of Web Publication10-Apr-2019

How to cite this article:
Sood N, Soin N. Superficial acral fibromyxoma: A cytohistopathological correlation in a recurrent, nonperiungual, acral lesion. Indian J Pathol Microbiol 2019;62:348-50

How to cite this URL:
Sood N, Soin N. Superficial acral fibromyxoma: A cytohistopathological correlation in a recurrent, nonperiungual, acral lesion. Indian J Pathol Microbiol [serial online] 2019 [cited 2022 Oct 1];62:348-50. Available from:

Dear Editor,

Superficial acral fibromyxoma (SAFM) was initially described by Fetsch et al. in 2001. It is an uncommon, solitary, slow growing soft tissue tumor affecting ungual and periungual regions of hands and toes in adults. It is more common in males with a mean age of 43 years at diagnosis.[1],[2]

Due to morphological and immunohistochemical overlap with other myxoid lesions cytological diagnosis is difficult in cases where the location is not classic.[3]

A 38-year-old male presented with a recurrent firm mass on the dorsum of right thumb away from nail bed. The patient was referred with the diagnosis of ganglion cyst. Fine needle aspiration cytology (FNAC) yielded thick mucoid material showing few scattered and loosely clustered spindle to stellate cells with fibrillary stroma in myxoid background. [Figure 1]. It was reported as low grade fibromyxoid lesion with suggestion of SAFM. The excised specimen was non-encapsulated, grey white 2 × 1 cm and gelatinous. On microscopy, there was multilobular configuration with predominant hypocellular areas showing benign stellate cells, occasional mast cells in an abundant myxoid material. Periphery of the lesion showed vascular proliferation and sclerotic bands. There was no atypia or mitotic figures [Figure 2]a and [Figure 2]b. Immunohistochemistry showed positivity for CD34 (focal), CD10 and CD99 while S100 was negative. [Figure 2]c and [Figure 2]d.
Figure 1: (a) Smear showing loosely scattered spindle cells in myxoid background (×40, H and E). (b) Smears showing stellate cells (×100, H and E). (c and d) Smear showing fibrillary stroma with entangled spindle cells (×400, H and E)

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Figure 2: (a) Section showing lobular configuration and alternating cellular and hypocellular areas (×100, H and E). (b) Section showing peripheral vascular proliferation (×100, H and E). (c) Section showing focal CD34 positivity (×100, IHC). (d) Section showing CD10 positivity. (×100, IHC)

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Cytological diagnosis of this entity is challenging with a very few case reports from Indian subcontinent. First case was reported by Sinha A et al. from a patient with subungual globular swelling on fifth toe.[4]

Subsequent case was reported by Raghupathi DS in a patient with lesion on great toe.[3]

Grossly SAFMs are unencapsulated, nodular masses ranging from 0.6 to 5 cm with solid or gelatinous cut surface.[2]

Microscopy reveals spindle and stellate cells in storiform pattern or in fascicles in loose myxoid/myxocollagenous background. Proliferating micro vasculature is common. Mitotic activity and significant nuclear pleomorphism are uncommon. On immunohistochemistry, the tumor cells are invariably CD99, CD34, CD10 and vimentin positive while negative for S100, desmin, alpha Smooth Muscle Actin (SMA), keratins and Glial Fibrillary Acid Protein (GFAP) with variable Epithelial Membrane Antigen (EMA) expression.[2]

Superficial angiomyxoma presents with similar features but has no predilection for digital or subungual sites. They are lobulated, lack alternating cellular and hypo cellular areas and have thin branching vessels, entrapped epithelial elements and a rich neutrophilic infiltrate, which was missing in this case. The tumor cells show expression of CD34, SMA (focal) and desmin.[2],[3],[5]

Myxoid neurofibroma can be distinguished from SAFMs by their neural morphology, lack of vasculature, more fibrillary collagen and S100 positivity, clearly lacking in this case.[2],[4],[5]

Low grade fibromyxoid sarcomas need to be ruled out because of their aggressive course. They comprise of bland spindle cells with alternating fibrous and myxoid areas with small inconspicuous vessels. They are CD34 negative but MUC4positive.[5]

Myxofibrosarcomas can be differentiated from SAFM by tiny foci of pleomorphic spindle cells with prominent curvilinear vessels. The cells are positive for vimentin.[5]

Other differential diagnosis are myxomas, digital mucus cyst and cutaneous focal mucinosis, giant cell tumor of tendon sheath, glomus tumor, dermal nerve sheath myxoma myxoid dermatofibrosarcoma protuberans, sclerosing perineuroma and giant cell fibroblastoma.

Fibromyxoid lesions on unconventional sites need to be examined with care to rule out potentially malignant lesions.

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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Fetsch JF, Laskin WB, Miettinen M. Superficial acralfibromyxoma: aclinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes. Hum Pathol 2001;32:704-4.  Back to cited text no. 1
Ashby-Richardson H, Rogers GS, Stadecker MJ. Superficial acralfibromyxoma: An overview. Arch Pathol Lab Med 2011;135:1064-6.  Back to cited text no. 2
Raghupathi DS, Krishnamurthy J, Kakoti LM. Cytological diagnosis of superficial acralfibromyxoma: A case report. J Cytol 2015;32:39-41.  Back to cited text no. 3
[PUBMED]  [Full text]  
Sinha A, Phukan JP, Bandyopadhyay G, Sengupta S, Saha R. Superficial AcralFibromyxoma: A rare tumor diagnosed by cytology. Iranian J Pathol 2013;8:59-63.  Back to cited text no. 4
Riddle ND, Gardner JM. The pale blue slide: Avoiding myx-ups and mishaps in cutaneous myxoid tumors. Diagn Histopathol 2016;22:152-66.  Back to cited text no. 5

Correspondence Address:
Navmeet Soin
Department of Pathology, Deen Dayal Upadhyay Hospital (Government of NCT), New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_238_18

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