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Head and Neck Pilomatrixoma in Children
Angelique Danielson-Cohen, MD;
Samuel J. Lin, MD;
C. Anthony Hughes, MD;
Young H. An, MD;
John Maddalozzo, MD
Arch Otolaryngol Head Neck Surg. 2001;127:1481-1483.
ABSTRACT
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Objective To provide a review of the current information on the etiology, clinical
presentation, management, and outcome of pilomatrixoma of the head and neck
in children.
Design Retrospective review.
Setting A tertiary care pediatric center.
Patients Fifty-one pediatric patients with a diagnosis of pilomatrixoma of the
head and neck.
Intervention All patients underwent excision of pilomatrixoma from January 1997 to
March 1999. A total of 55 tumors were studied.
Results A preponderance of girls (n = 36; 71%) presented with this condition.
The average age at diagnosis was 5.7 years, and the average size of the lesion
was 1 cm. The skin of the cheek and the periorbital area were the most commonly
involved sites. Only 27 lesions (49%) had a correct preoperative diagnosis.
Two (4%) of 55 tumors recurred after complete surgical excision.
Main Outcome Measures The age and sex of the patient, preoperative diagnosis, time elapsed
before diagnosis, site and size of the tumor, length of follow-up, presence
of multiple or previous pilomatrixomas, and recurrence.
Conclusions Preoperative diagnosis may be improved with increased awareness of pilomatrixoma,
a common, benign skin tumor in children. Clinical findings will aid in an
accurate diagnosis. Recurrence after complete local excision is rare.
INTRODUCTION
IN 1880, Malherbe and Chenantais1 first
described pilomatrixomas, then thought to arise from sebaceous glands2 and called calcifying epitheliomas
of Malherbe. In 1961, Forbis and Helwig3
proposed the term pilomatrixoma to avoid a connotation
of malignancy. At this time, the origin of pilomatrixoma from hair matrix
cells was discovered.3
Pilomatrixomas commonly occur in children and most frequently in the
head and neck region. They are the second most commonly excised superficial
masses in children after epidermoid cysts and excluding lymph nodes.4 However, pilomatrixomas are frequently misdiagnosed
and/or missed in the differential diagnosis. Surgical removal is curative.
Recurrence after complete local excision is rare. Malignancy has been rarely
reported.
The lack of discussion of pilomatrixoma in the otolaryngology literature
despite its frequent occurrence prompted this review. The objective of this
study is to provide information on the etiology, clinical presentation, management,
and outcome of pilomatrixoma of the head and neck in children. We report the
results of our own study, in which we analyzed data from 51 children with
a diagnosis of pilomatrixoma of the head and neck, a total of 55 tumors; and
we provide a review of the literature. The clinical presentation, tumor behavior,
treatment, and outcome of pilomatrixoma are reviewed.
RESULTS
There were 51 patients with a total of 55 pilomatrixomas of the head
and neck region; 36 (71%) were girls, 15 (29%) were boys (ratio, 2.4:1). Patient
age at diagnosis varied from 8 months to 16 years, with 35 patients (69%)
presenting with the condition at younger than 7 years; 37 (72%) were diagnosed
within 6 months of developing the lesion.
Figure 1 shows the sites of
pilomatrixoma occurrence. Seventeen lesions (31%) occurred around the eye,
including the brow and periorbital area, 15 (27%) in the cheek region, and
7 (13%) in the preauricular region.
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Figure 1. Areas of occurrence of 55 pilomatrixomas.
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The varied preoperative diagnoses are depicted in Figure 2. A correct preoperative diagnosis was made 49% of the time.
The most common misdiagnosis was a dermoid. Six patients (12%) reported tenderness
and 5 (9%) reported a history of trauma in the region of the tumor.
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Figure 2. Preoperative diagnosis of masses
(N = 55) prior to excision and pathological examination (number of masses/percentage
of total pilomatrixoma tumors).
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All tumors were slow growing. A bluish hue or telangiectasia was found
in 17 lesions (31%); 5 patients (10%) had multiple pilomatrixomas at the time
of diagnosis, including areas outside of the head and neck region. Three patients
(6%) had a history of a previous pilomatrixoma. All lesions were surgically
excised (1 patient underwent attempted removal with liquid nitrogen and experienced
recurrence; surgical excision was then performed). Three lesions (6%) recurred
overall; after complete surgical excision, 2 lesions (4%) recurred, which
is similar to the literature.3
COMMENT
Pilomatrixomas are of ectodermal origin and arise from the outer root
sheath cell of the hair follicle.3, 5
They arise in the lower dermis and form a connective tissue capsule. Shadow
or ghost cells, with a central unstained area representing a shadow of a lost
nucleus, are common.6 (Figure 3). Basaloid cells have a round or elongated basophilic nucleus
and scant cytoplasm at the periphery of epithelial islands6
(Figure 4). Calcium deposition and
a foreign body reaction commonly occur,7 and
ossification has been reported.6
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Figure 3. Pilomatrixoma showing shadow cells
with naked nuclei and abundant cytoplasm (hematoxylin-eosin, original magnification
x200).
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Figure 4. Pilomatrixoma showing inferior
and central basaloid cells becoming larger islands of shadow cells (hematoxylin-eosin,
original magnification x100).
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The diagnosis of pilomatrixoma can be made clinically if the characteristics
of the tumor are known (Figure 5):
pilomatrixoma lesions slide freely over the underlying area. Graham and Merwin8 described the "tent sign," elicited by stretching
the skin over the pilomatrixoma tumor to feel the irregular surface of the
mass. There is no associated lymphadenopathy. A blue discoloration may be
seen. We have found the most common locations of pilomatrixoma to be the cheek
area and, as Orlando et al7 have noted, the
periorbital area. There may be a history of regional trauma prior to developing
the tumor, as was found in 5 (9%) of our patients (which matches the results
of the literature3). The significance of this
is unknown.
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Figure 5. Preoperative photograph of a recurrent
pilomatrixoma of the cheek.
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The pilomatrixoma tumor commonly occurs in the head and neck region
of children. Our age of presentation was similar to that in the literature,
with most patients presenting at younger than 7 years.3, 7
We found multiple tumors in 5 (10%) of our patients, which is higher than
the 2% to 3.5% reported in the literature.6
Multiple pilomatrixomas have been associated with Gardner syndrome, Steinert
disease, myotonic dystrophy, and sarcoidosis,3, 9-11
although these diseases were not found in our patients. We also found a 71%
female preponderance (36 girls vs 15 boys) that has not been found by others.3, 7
The differential diagnosis of pilomatrixoma is varied. Pilomatrixoma
should be differentiated from epidermal and dermoid cysts. Epidermal cysts
are firm, round, and mobile, and they have normal overlying skin. They also
present in an older age group, both adolescents and adults.7
Dermoid cysts are firmly attached to underlying tissue and are often found
in children. Neither presents with irregular nodules on the skin as pilomatrixoma
does.
Diagnostic tests and imaging studies are often unnecessary in the workup
of a superficial, benign skin lesion such as pilomatrixoma. However, tests
are sometimes done to exclude the diagnosis of malignancy or to determine
the depth of a lesion. Pilomatrixoma in the parotid or preauricular region
may be a diagnostic dilemma. If the mass is of branchial or parotid origin,
careful dissection from the parotid gland may be necessary, and preoperative
knowledge of the extent of the lesion may be helpful.
Fine-needle aspiration may reveal the presence of ghost cells, basaloid
cells, and/or calcium deposition in the mass, which findings are diagnostic
of pilomatrixoma.12 However, without the presence
of ghost cells in the aspirate, the diagnosis may be misleading.12
On routine soft tissue radiographs, which have fallen out of use since advent
of superior imaging techniques for soft tissue masses, pilomatrixoma may appear
as a regularly contoured mass with homogeneous speckles and occasional dense
foci of calcification.13 Fink and Berkowitz5 found ultrasound to be particularly helpful in children
to determine the relation of the mass to the parotid. This method does not
require sedation or anesthesia. Computed tomography and magnetic resonance
imaging will add greater detail to the surrounding structures and depth of
the lesion, but these imaging techniques are costly and may require sedation
or the use of anesthesia in children.
Malignant pilomatrixoma has never been reported in children and was
not found in our series. Black et al6 report
the clinical behavior of pilomatrix carcinoma in adults to resemble that of
basal cell carcinoma in its potential to metastasize. Treatment is wide local
excision. Reconstruction may be best deferred for 1 year to allow for close
observation for recurrence. The role of radiation is unknown owing to the
small number of reported cases, but may help in locoregional control.6
The treatment of choice and standard therapy for benign pilomatrixoma
is complete surgical excision. Occasionally, overlying skin will need to be
excised secondary to tumor adherence to the dermis. Morales and McGoey14 have advocated incision and curettement for cosmetic
preservation in large tumors or for those in exposed areas. No recurrence
was found in their series. Our series noted that 2 tumors (4%) recurred after
complete surgical excision. One lesion in our study failed to respond to liquid
nitrogen therapy and required surgical excision.
AUTHOR INFORMATION
Accepted for publication July 17, 2001.
Presented as a poster at the 15th Annual Meeting of the American Society
of Pediatric Otolaryngology, Orlando, Fla, May 16, 2000.
Corresponding author and reprints: John Maddalozzo, MD, Division
of Pediatric Otolaryngology, Children's Memorial Hospital, 2300 Children's
Plaza, Box 25, Chicago, IL 60614 (e-mail: jmaddalozzo{at}childrensmemorial.org).
From the Departments of Otolaryngology–Head and Neck Surgery,
University of Illinois Hospital at Chicago (Drs Danielson-Cohen and An); and
Northwestern Memorial Hospital (Dr Lin), and the Divisions of Otolaryngology
(Dr Hughes) and Pediatric Otolaryngology (Dr Maddalozzo), Children's Memorial
Hospital, Chicago, Ill.
REFERENCES
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1. Malherbe A, Chenantais J. Note sur l'épithélioma calcifiédes glandes sébacées. Prog Med. 1880;8:826-837.
2. Julian CG, Bowers PW. A clinical review of 209 pilomatricomas. J Am Acad Dermatol. 1998;39:191-195.
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3. Forbis R, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol. 1961;83:606-617.
4. Knight PJ, Reiner CB. Superficial lumps in children: what, when and why? Pediatrics. 1983;72:147-153.
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5. Fink AM, Berkowitz RG. Sonography in preauricular pilomatrixoma of childhood. Ann Otol Rhinol Laryngol. 1997;106:167-169.
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6. Black SJ, Marble BF, Vuitch F. Multiple giant pilomatrix carcinomas of the head and neck. Otolaryngol Head Neck Surg. 1993;109:543-547.
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7. Orlando RG, Rogers GL, Bremer DL. Pilomatricoma in a pediatric hospital. Arch Ophthalmol. 1983;101:1209-1210.
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8. Graham JL, Merwin CF. The tent sign of pilomatricoma. Cutis. 1978;22:577-580.
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9. Urvoy M, Legall F, Toulemont PJ, Chevrant-Breton J. Multiple pilomatricoma. Apropos of a case. J Fr Ophthalmol. 1996;19:464-466.
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10. McCulloch TA, Singh S, Cotton DWK. Pilomatrix carcinoma and multiple pilomatrixomas. Br J Dermatol. 1996;134:368-371.
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11. Harper P. Calcifying epithelioma of Malherbe: association with myotonic dystrophy. Arch Dermatol. 1972;106:41-44.
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12. Domanski HA, Domanski AM. Cytology of pilomatrixoma (calcifying epithelioma of Malherbe) in fine
needle aspirates. Acta Cytol. 1997;41:771-777.
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13. Haller JO, Kassner G, Ostrowitz A, Kottmeier K, Pertschuk LP. Pilomatrixoma (calcifying epithelioma of Malherbe): radiographic features. Radiology. 1977;123:151-153.
ABSTRACT
14. Morales A, McGoey J. Pilomatricoma: treatment by incision and curettement. J Am Acad Dermatol. 1980;2:44-46.
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