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Eyebrow madarosis reflecting an intradermal neoplasm: pleomorphic adenoma, a rare brow tumour

Published:April 28, 2016DOI:https://doi.org/10.1016/j.jcjo.2016.01.013
      Pleomorphic adenoma is a neoplasm named for its mixture of disparate tissue elements, including branching double-layered ductules within a fibroid, myxoid, cartilaginoid, and (rarely) adipocytic stroma.
      • Palioura S.
      • Jakobiec F.A.
      • Zakka F.R.
      • Iwamoto M.
      Pleomorphic adenoma (formerly chondroid syringoma) of the eyelid margin with a pseudocystic appearance.
      A rare cutaneous tumour presumably arising from apocrine or eccrine sweat ducts, it has been described in the eyelid, where it may occupy various locations, including the sub-brow region.
      • Palioura S.
      • Jakobiec F.A.
      • Zakka F.R.
      • Iwamoto M.
      Pleomorphic adenoma (formerly chondroid syringoma) of the eyelid margin with a pseudocystic appearance.
      • Mandeville J.T.
      • Roh J.H.
      • Woog J.J.
      • et al.
      Cutaneous benign mixed tumor (chondroid syringoma) of the eyelid: clinical presentation and management.
      Well-recognized tumours with identical histology may arise from the ductules of the major salivary glands and all portions of the lacrimal gland.
      • Font R.
      • Croxatto J.O.
      • Rao N.A.
      Tumors of the lacrimal gland.
      We report a pleomorphic adenoma of the brow that presented with the unusual symptom of focal brow loss.
      A healthy 77-year-old male complained of hair loss on the temporal third of the left eyebrow. Examination disclosed a subtly elevated, nontender, faintly erythematous subcutaneous mass in that location (Fig. 1). Excisional biopsy including overlying skin yielded a mass measuring 0.7 cm × 0.2 cm × 0.3 cm. Histology showed a well-circumscribed, nonencapsulated intradermal tumour consisting of branching and nonbranching strands of tubuloalveolar structures arranged among chondroid and adipocytic zones (Fig. 2A). The tubules were composed of a double layer of epithelial cells, the inner layer of which stained positively with epithelial immunostains AE1/AE3, EMA, and CAM5.2 (spotty) as well as gross cystic fluid disease protein (GCDFP-15), an apocrine marker. The outer layer was positive for S100 and NSE but not SMA or GFAP. Ki67 showed a low proliferative rate (<5%).
      Fig. 1
      Fig. 1Hair loss and tumour of the temporal portion of the left brow (arrow). The pigmented eyelid lesion is a seborrheic keratosis.
      Fig. 2
      Fig. 2(A) Tumour consists of branching, double-layered tubuloalveolar structures amidst adipose tissue (asterisk) and chrondroid tissue (arrowhead). Inset shows a double layer of cuboidal cells (hematoxylin–eosin, original magnification ×200, inset ×400). (B) Immunohistochemistry highlights adluminal layer of tubules for AE1/AE3 pancytokeratin stain (upper left) and CAM 5.2 low-molecular-weight keratin (upper right). Outer layer of cuboidal cells stains positively for neuron-specific enolase (NSE) (lower left). Inner layer is positive for gross cystic disease fluid protein-15 (GCDFP-15) (lower right) (immunperoxidase reaction, diaminobenzidine chromogen, ×400).
      Formerly designated “chondroid syringoma” and “cutaneous benign mixed tumour,” the current preferred terminology of this rare cutaneous neoplasm is “pleomorphic adenoma” implying morphologic variety that includes epithelial and mesenchymal portions.
      • Palioura S.
      • Jakobiec F.A.
      • Zakka F.R.
      • Iwamoto M.
      Pleomorphic adenoma (formerly chondroid syringoma) of the eyelid margin with a pseudocystic appearance.
      Histology shows a biphasic, variable architecture containing epithelial tubules set in a fibrous, myxoid, cartilaginoid, and even an unusual adipocytic stroma as in the current case. Immunohistochemisty shows a different staining pattern for the inner tubular cells compared with the outer layer. As there are no characteristic clinical features, diagnosis relies upon excisional biopsy. Malignant transformation has been described, especially after incomplete excision, paralleling the experience in the lacrimal and salivary glands.
      • Gündüz K.
      • Demirel S.
      • Heper A.O.
      • Günalp I.
      A rare case of atypical chondroid syringoma of the lower eyelid and review of the literature.
      The differential diagnosis of eyebrow madarosis is extensive, including common localized disorders such as seborrheic dermatitis, malignancies (basal and squamous cell carcinoma), or components of systemic diseases (alopecia areata, discoid lupus erythematosis, Vogt–Koyanagi–Harada syndrome, sarcoidosis, leprosy, syphilis, cutaneous T-cell lymphoma).
      • Kumar A.
      • Karthikeyan K.
      Madarosis: a marker of many malidies.
      Focal brow infarction may provide a clue to the diagnosis of giant cell arteritis. Drugs such as retinoids, heparin, anticonvulsants, angiotensin-converting enzyme (ACE) inhibitors, and androgens may trigger telogen hair shedding and madarosis.
      • Tosti A.
      • Pazzaglia M.
      Drug reactions affecting hair: diagnosis.
      Hair loss after systemic chemotherapy is well known. Patients may not admit to trichotillomania, a disorder involving the plucking of hairs.
      • Smith J.R.
      Trichotillomania: ophthalmic presentation.
      The approach to brow loss may be challenging and should involve consideration of the systemic medical status, including medication usage, and possible psychiatric evaluation. Focal and asymmetric brow loss merits suspicion for an underlying tumour
      • Kumar S.
      Rapidly growing pilomatrixoma on eyebrow.
      as in the current case. Complete surgical excision should always be attempted.

      References

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        Pleomorphic adenoma (formerly chondroid syringoma) of the eyelid margin with a pseudocystic appearance.
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