Clinicopathologic Conference| Volume 134, ISSUE 5, P505-512, November 2022

Download started.


A 33-year-old man with a rapidly growing lump on the dorsal tongue

Published:February 22, 2022DOI:
      A 33-year-old male professional singer presented to his dentist with a rapidly growing lump on his tongue in October 2019. He was a non-smoker and had no significant medical or family history. The patient was initially not aware of the presence of the lesion until he felt that he may have burnt the site with a hot meal. On examination, there was a 3 × 2 cm circumscribed, firm, broad-based and slightly fixed submucosal lump on the right posterior dorsal tongue. The lump was covered with normal appearing mucosa with a prominent network of superficial blood vessels (Figure 1). No mucosal ulceration or other lesions were detected on examination. The patient did not report any symptoms or loss of function.
      Fig 1
      Fig. 1Clinical features. (A) Frontal view of unilateral, firm, raised, broad-based submucosal lesion involving the right posterior dorsal tongue without crossing the mid-line. (B) The lump was covered with normal appearing mucosa with a prominent network of superficial blood vessels and no evidence of ulceration.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


        • Kuriakose MA
        • Chakrabarti S
        • Cheong SC
        • Kowalski LP
        • Novaes Pinheiro T
        • Farah CS
        Head and neck tumors.
        in: Farah C Balasubramaniam R McCullough M Contemporary Oral Medicine. Springer International Publishing, Cham, Switzerland2019
        • Goldblum JR
        • Folpe A
        • Weiss SW.
        Enzinger and Weiss's Soft Tissue Tumors.
        6th ed. Saunders/Elsevier, Philadelphia, PA2014
        • Beham A
        • Badve S
        • Suster S
        • Fletcher CD.
        Solitary myofibroma in adults: clinicopathological analysis of a series.
        Histopathology. 1993; 22: 335-341
        • Williams JO
        • Schrum D.
        Congenital fibrosarcoma; report of a case in a newborn infant.
        AMA Arch Pathol. 1951; 51: 548-552
        • Stout AP
        Juvenile fibromatoses.
        Cancer. 1954; 7: 953-978
        • Chung EB
        • Enzinger FM.
        Infantile myofibromatosis.
        Cancer. 1981; 48: 1807-1818
        • Daimaru Y
        • Hashimoto H
        • Enjoji M.
        Myofibromatosis in adults.
        Am J Surg Pathol. 1989; 13: 859-865
        • WHO Classification of Tumours Editorial Board
        Soft Tissue and Bone Tumors.
        5th ed. WHO Classification of Tumors, Lyon, France2020 (Vol. 3)
        • Smith A
        • Orchard D.
        Infantile myofibromatosis: two families supporting autosomal dominant inheritance.
        Australas J Dermatol. 2011; 52: 214-217
        • Kulkarni K
        • Desai S
        • Grundy P
        • Sergi C.
        Infantile myofibromatosis: report on a family with autosomal dominant inheritance and variable penetrance.
        J Pediatr Surg. 2012; 47: 2312-2315
        • Martignetti JA
        • Tian L
        • Li D
        • et al.
        Mutations in PDGFRB cause autosomal-dominant infantile myofibromatosis.
        Am J Hum Genet. 2013; 92: 1001-1007
        • Smith MH
        • Reith JD
        • Cohen DM
        • Islam NM
        • Sibille KT
        • Bhattacharyya I.
        An update on myofibromas and myofibromatosis affecting the oral regions with report of 24 new cases.
        Oral Surg Oral Med Oral Pathol Oral Radiol. 2017; 124: 62-75
        • Vered M
        • Allon I
        • Buchner A
        • Dayan D.
        Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity. II. Myofibroma and myofibromatosis of the oral soft tissues.
        J Oral Pathol Med. 2007; 36: 304-314
        • Mentzel T
        • Calonje E
        • Nascimento AG
        • Fletcher CD.
        Infantile hemangiopericytoma versus infantile myofibromatosis study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions.
        Am J Surg Pathol. 1994; 18: 922-930
        • Linos K
        • Carter JM
        • Gardner JM
        • Folpe AL
        • Weiss SW
        • Edgar MA.
        Myofibromas with atypical features: expanding the morphologic spectrum of a benign entity.
        Am J Surg Pathol. 2014; 38: 1649-1654
        • Jordan RC
        • Regezi JA.
        Oral spindle cell neoplasms: a review of 307 cases.
        Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003; 95: 717-724
        • Foss RD
        • Ellis GL.
        Myofibromas and myofibromatosis of the oral region: a clinicopathologic analysis of 79 cases.
        Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000; 89: 57-65
        • Mentzel T
        • Dei Tos AP
        • Sapi Z
        • Kutzner H
        Myopericytoma of skin and soft tissues: clinicopathologic and immunohistochemical study of 54 cases.
        Am J Surg Pathol. 2006; 30: 104-113
        • Hung YP
        • Fletcher CDM
        Myopericytomatosis. Clinicopathologic analysis of 11 cases with molecular identification of recurrent PDGFRB alterations in myopericytomatosis and myopericytoma.
        Am J Surg Pathol. 2017; 41: 1034-1044
        • Agaimy A
        • Bieg M
        • Michal M
        • et al.
        Recurrent somatic PDGFRB mutations in sporadic infantile/solitary adult myofibromas but not in angioleiomyomas and myopericytomas.
        Am J Surg Pathol. 2017; 41: 195-203
        • Dahlén A
        • Fletcher CD
        • Mertens F
        • et al.
        Activation of the GLI oncogene through fusion with the beta-actin gene (ACTB) in a group of distinctive pericytic neoplasms: pericytoma with t(7;12).
        Am J Pathol. 2004; 164: 1645-1653
        • Agaram NP
        • Zhang L
        • Sung YS
        • et al.
        GLI1-amplifications expand the spectrum of soft tissue neoplasms defined by GLI1 gene fusions.
        Mod Pathol. 2019; 32: 1617-1626
        • Antonescu CR
        • Sung YS
        • Zhang L
        • Agaram NP
        • Fletcher CD.
        Recurrent SRF-RELA fusions define a novel subset of cellular myofibroma/myopericytoma: a potential diagnostic pitfall with sarcomas with myogenic differentiation.
        Am J Surg Pathol. 2017; 41: 677-684
        • Karanian M
        • Kelsey A
        • Paindavoine S
        • et al.
        SRF fusions other than with RELA expand the molecular definition of SRF-fused perivascular tumors.
        Am J Surg Pathol. 2020; 44: 1725-1735
        • Dachy G
        • de Krijger RR
        • Fraitag S
        • et al.
        Association of PDGFRB mutations with pediatric myofibroma and myofibromatosis.
        JAMA Dermatol. 2019; 155: 946-950
        • Arts FA
        • Sciot R
        • Brichard B
        • et al.
        PDGFRB gain-of-function mutations in sporadic infantile myofibromatosis.
        Hum Mol Genet. 2017; 26: 1801-1810