The Journal of Foot & Ankle Surgery xxx (2017) 14

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The Journal of Foot & Ankle Surgery

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Case Reports and Series

Onychomatricoma: A Rare and Potentially Underreported Tumor

of the Nail Matrix
Calvin J. Rushing, DPM 1, Roman Ivankiv, DPM 1, Neal M. Bullock, DPM 2,
Diana E. Rogers, DPM 3, Steven M. Spinner, DPM 4
1
Resident, Graduate Medical Education, Westside Regional Medical Center, Plantation, FL
2
Attending Physician, Westside Regional Medical Center, Plantation, FL
3
Research Director, Westside Regional Medical Center, Plantation, FL
4
Residency Director, Westside Regional Medical Center, Plantation, FL

a r t i c l e i n f o a b s t r a c t

Level of Clinical Evidence: 4 Onychomatricoma is a rare benign neoplasm of the nail matrix rst described by Baran and Kint in 1992. Fewer
than 80 cases of onychomatricoma have been described in the literature, 15 of which were initially mis-
Keywords:
nail diagnosed and treated as onychomycosis. We present the case of a 66-year-old male with thickening and
neoplasm linear xanthonychia of the hallux nail plate secondary to an onychomatricoma misdiagnosed as onychomy-
onychomycosis cosis. Following biopsy for histopathologic analysis, the lesion and proximal nail matrix were surgically
onychomatricoma excised. At 12 months post-excision, the patient remains asymptomatic without evidence of recurrence. The
tumor purpose of the present case report is to make foot and ankle surgeons more cognizant of the pathology,
highlight the nonspecic clinical and radiologic ndings, and emphasize the importance of interdisciplinary
communication for an accurate clinicopathologic correlation and diagnosis of the lesion. Although rare,
onychomatricoma should be considered in the differential diagnosis for patients presenting with onycho-
mycosis failing to respond to antimycologic treatment. The clinical index of suspicion for onychomatricoma
should increase when only a singular dystrophic nail is involved. Following diagnostic conrmation by his-
topathology, complete surgical excision is the treatment of choice.
2017 by the American College of Foot and Ankle Surgeons. All rights reserved.

Onychomatricoma (OM) is a rare benign neoplasm of the nail matrix
rst described by Baran and Kint (1) in 1992 and later coined by Haneke
and Franken (2). The broepithelial lesion is characterized by nger-like
projections that penetrate the nail plate of the involved digit, with a
predominance of 2:1 in the nger. Fewer than 80 cases of OM have been
described in the literature, 15 of which were initially misdiagnosed
(Table) and treated as primary onychomycosis (213). The classic clinical
features (3) of OM include thickening of the nail plate, linear xantho-
nychia, transverse overcurvature, and splinter hemorrhages, which may
mimic the clinical presentation of other nail pathology (217). Moreover,
the penetrating projections of OM render the nail susceptible to invasion
by mycoses, and coincident cases of onychomatricoma and onychomy-
cosis have been documented (3,14,17), further compounding the diag-
nostic dilemma associated with lesion presentation.
Onychomycosis is responsible for 50% of all nail disease and affects
approximately 14% of the North American population annually (18).
Financial Disclosure: None reported.
Conict of Interest: None reported.
Address correspondence to: Calvin J. Rushing, DPM, Graduate Medical Education,
Westside Regional Medical Center, 8201 West Broward Boulevard, Plantation, FL 33324.
E-mail address: calvin.rushing@mymail.barry.edu (C.J. Rushing).
Although oral antifungal medications are commonly prescribed for
onychomycosis treatment, they are not without a myriad of side ef-
fects. Thus, their use should be judicious and reserved for patients
with conrmed onychomycosis documented by histopathologic ex-
amination, rather than by clinical examination alone. We present the
case of a 66-year-old male with OM of the left hallux nail, mis-
diagnosed and treated as onychomycosis with topical and oral anti-
fungal medications for 4 years without resolution.
Case Report
A 66-year-old male with no signicant medical history presented
to our ofce (N.M.B.) in April 2016 with cosmesis concerns and mild
discomfort at the medial border of his left hallux nail plate. The pa-
tient related a history of an onychomycotic nail that was recalcitrant
to topical and oral antifungal agents prescribed by his primary care
physician for the previous 4 years. The patient denied any history of
trauma or an inciting event and reported the gradual thickening of the
nail during the previous decade. He had become increasingly frus-
trated and insecure about the appearance of the nail and now
requested it be removed in total. At the initial evaluation, the range of

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2 C.J. Rushing et al. / The Journal of Foot & Ankle Surgery xxx (2017) 14

irregularity with speculation versus crenulation of the supercial

Table aspect of the nail bed, with edema to the bed and tuft of the distal
Cases of onychomatricoma initially misdiagnosed as primary onychomycosis and duration phalanx of the left great toe suspicious for OM.
between initial presentation of nail plate changes and denitive diagnosis and treatment
Wide local excision of the lesion was performed with the patient in
Investigator Misdiagnosed Interval Before the supine position under intravenous sedation on June 16, 2016. An
Cases (n) Diagnosis ipsilateral ankle tourniquet was used after exsanguination of the ex-
Kallis et al (3), 2016 2 2 mo, 2 y tremity by elevation and a digital anesthetic H-block with 3 mL of a
Joo et al (4), 2016 1 8y 1:1 mixture of 1% lidocaine and 0.5% Marcaine. Intraoperatively, a
Graves et al (5), 2015 1 13 y
Poojary et al (6), 2015 1 2y
esh-colored tumor with residual tumoral projections emerging from
Ozu turk Durmaz et al (7), 2013 1 15 y the nail matrix was visualized (Fig. 4). The lesion was resected en bloc,
Ferna ndez-Sanchez et al (8), 2012 1 3y with normal nail matrix proximal to the lesion included to prevent
Durrant et al (9), 2012 1 2y recurrence. Repeat histopathologic examination conrmed the diag-
Soto et al (10), 2009 3 1020 y
nosis of OM, and repeat periodic acid-Schiff staining, Gomoris
Khelifa et al (11), 2008 1 5y
Estrada-Chavez et al (12), 2007 1 3y methenamine silver staining, and fungal culture again yielded nega-
Van Holder et al (13), 1999 1 20 y tive results for mycoses. The postoperative course was uneventful,
Haneke et al (2), 1995 1 8y and at 12 months post wide local excision, the patient remains
asymptomatic without evidence of recurrence.

motion for all muscle groups of the foot and ankle were within normal
limits, and the results of the neurovascular examination were normal,
without numbness or paresthesia.
Physical examination of the left hallux nail plate revealed longi-
tudinal thickening and linear xanthonychia, mimicking onychomy-
cosis. No erythema, edema, seropurulent exudate, granulation tissue,
or chronic induration of the nail fold was present. The nail plate
morphology of the other digits was notably normal, without dystro-
phic changes visualized. Initial nail plate biopsy for mycoses yielded
negative results using periodic acid-Schiff staining, Gomoris methe-
namine silver staining, and fungal culture (Fig. 1).
Avulsion of the nail revealed bizarre nger-like projections
arising from the nail matrix, with cavities in the proximal portion of
the nail plate, consistent clinically with OM (Fig. 2). Histopathologic
examination of the specimen failed to provide evidence of OM on the
initial review. After a discussion with the dermatopathologist, a
second review was performed, and an addendum was added, doc-
umenting papillary fragments of the nail matrix lined by squamous
epithelium consistent with OM (Fig. 3). Radiographic examination of
the left foot revealed no underlying osseous involvement linked to
OM, and ultrasound imaging failed to provide diagnostic evidence of
the pathology. A preoperative magnetic resonance imaging scan
without contrast of the left foot also failed to provide evidence of OM
on the initial review, reporting sesamoiditis, chronic fracture of the
tibial sesamoid, a Heuters neuroma, and spurring with edema
around the rst metatarsophalangeal joint. After a discussion with
the radiologist, a second review revealed a single axial short tau
inversion recovery magnetic resonance image demonstrating
Discussion
OM is a benign broepithelial lesion of the nail matrix rarely re-
ported in the literature. This may be because of fragmented histo-
pathologic specimens, a lack of interdisciplinary communication for
an accurate clinicopathologic correlation, or the obscurity of the
lesion. The purpose of the present case presentation is to highlight
OM as a potential etiology for nail unit dystrophy and the diagnostic
dilemma associated with the lesions presentation. Fewer than 80
documented cases have been reported, 15 of which were initially
misdiagnosed (213) and treated as primary onychomycosis (Table).
Most presentations have affected the ngernails of middle-age
women; hence, clinical suspicion in the toenails has been low. How-
ever, with clinical features presenting similar to those of onychomy-
cosis, misdiagnosis, inappropriate treatment, and an underreporting
of the lesion in the lower extremity may not be uncommon.
The clinical features of OM result from nger-like projections that
penetrate the nail plate of the involved digit causing thickening of the
nail plate, linear xanthonychia, transverse overcurvature, and splinter
hemorrhages. The projections can produce eye comb-like cavities
termed woodworm cavities when viewed in the coronal plane of the
nail plates free margin and render the nail plate susceptible to invasion
by mycoses (19). Coincident cases of OM and onychomycosis have been
documented (3,14,17), as have pigmented variants presenting as lon-
gitudinal melanonychia, and subungual melanoma (1416). Other en-
tities in the differential diagnosis include brokeratoma, ungual
broma, subungual verruca vulgaris, Bowens disease, and
osteochondroma.

Fig. 1. No evidence of mycoses on histopathologic examination of the patients nail clippings. (A) Periodic acid-Schiff staining (original magnication  10). (B) Gomoris methenamine
silver staining (original magnication  10).
 C.J. Rushing et al. / The Journal of Foot & Ankle Surgery xxx (2017) 14
Fig. 2. Characteristic cavities of onychomatricoma in the proximal portion of the nail plate
following complete nail avulsion.
In the diagnostic assessment of OM, clinical features alone cannot
be used to reliably diagnose the lesion. Additional diagnostic methods
include radiography, ultrasonography, magnetic resonance imaging,
dermoscopy, and histopathologic examination. Radiographic exami-
nation will demonstrate no underlying osseous involvement, and
ultrasound examination may reveal a hypoechogenic tumor in the
nail matrix with hyperechogenic nger-like projections (4). On
magnetic resonance imaging scans, the proximal portion of the lesion
involving the nail matrix appears low-signal intensity and the nger-
like projections distally high-signal intensity (20). Dermoscopy con-
tinues to become more widely used as a diagnostic tool clinically for
OM, owing to 2 characteristic ndings (3). The rst is at the nail plate,
where a jagged edge appearance of the proximal margin of the ony-
cholytic area, with spikes directed to the proximal nail fold and an
irregular pigmentation in the longitudinal striae, can be appreciated.
The second nding is at the free margin of the nail, where honey-
comb-like cavities of OM can be appreciated.
Histopathologic examination after biopsy (clippings, plate, tumor)
remains the reference standard for the diagnosis of OM (4). Histo-
pathologically, the proximal zone of OM lies deep to the proximal nail
fold and is characterized by overlying ungual protrusions and deep
epithelial invaginations. The distal zone of the OM corresponds to the
Fig. 3. Histopathology of onychomatricoma demonstrating papillary fragments of the nail matrix lined by squamous epithelium. (A) Original magnication  4. (B) Original magnication
 10.
3
Fig. 4. Intraoperative image of onychomatricoma with tumoral projections arising from
the nail matrix.
lunula and is characterized by broepithelial projections that perforate
the nail plate, originating from the nail matrix proper (21). Prior reports
have highlighted the importance of interdisciplinary communication
and an accurate clinicopathologic correlation for the diagnosis of OM,
because unoriented sectioning by the dermatopathologist can delay the
denitive diagnosis and treatment (16). Following diagnostic conr-
mation by histopathology, wide local excision is the treatment of choice
to prevent recurrence. To date, only a single case report has docu-
mented recurrence of OM after excision (22).
Most patients with OM experience slow growth of the tumor and
the absence of pain. Although the exact etiology of OM remains un-
known, nail plate trauma and onychomycosis could be predisposing
factors. It has been suggested that OM may represent a hamartoma
simulating the nail matrix structure, although immunohistochemical
staining for CD10 suggests a true neoplastic nature (23). Recently, a
possible association with the loss of the STIM1 (stromal interaction
molecule 1) and CTSC (cathepsin C) genes on chromosome 11 was
identied and might assist with future diagnoses (24). Although most
published studies have reported a 2:1 predominance of OM in the
ngernail (versus toenail), misdiagnosis is common, and the true
incidence in the lower extremity remains to be determined. As more
foot and ankle surgeons become cognizant of the pathology, the
number of documented case reports may increase.
In conclusion, OM remains a diagnostic challenge. Although rare, it
should be considered a differential diagnosis in patients with clinical
features of onychomycosis failing to respond to antimycologic
4 C.J. Rushing et al. / The Journal of Foot & Ankle Surgery xxx (2017) 14
treatment. The clinical index of suspicion for onychomatricoma
should increase when only a singular dystrophic nail is involved.
Following diagnostic conrmation by histopathology, complete sur-
gical excision is the treatment of choice.
Acknowledgments
The authors thank Scott Frier, Rosa M. Ferra, MD, and Douglas
Hornsby, MD, for their assistance in preparation of this report.
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