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Pilomatrix Carcinoma of the Head and Neck: Case Report and Literature Review

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Introduction: Pilomatrix carcinoma is a rare cutaneous malignancy of hair matrix cells. They exhibit an aggressive and infiltrative growth pattern and, despite treatment to the local disease, there is a high propensity for recurrence. The literature reports a poor prognosis associated with regional spread occurring with distant metastasis. The patient we report is alive at over two years post-operatively. With only one other case of a head and neck pilomatrix carcinoma ever reported in Canada, our report and review of the literature contributes to what little is known currently about the treatment of this malignancy. Case report: In previously reported cases, regional lymph node metastasis is always associated with distant metastatic disease on presentation, thus rendering the treatment palliative in nature. We present the unique case of a 63-year-old man with a pilomatrix carcinoma of the left parotid region. He had bilateral cervical lymph node spread but no detectable distant metastasis. The patient underwent a wide excision of the lesion with a radical parotidectomy, temporal bone resection, bilateral neck dissections, and post-operative radiation. Conclusion: The treatment of pilomatrix carcinomas involve extensive surgical management with wide margins. Post-operative radiation in patients without distant metastasis may decrease the risk of local recurrence.
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Licensee OAPL (UK) 2014. Creative Commons Attribution License (CC-BY)
FOR CITATION PURPOSES
: Liu CC, Hoy M, Matthews TW, Guggisberg K, Chandarana S. Pilomatrix carcinoma of the
head and neck: Case report and literature review. Head Neck Oncol 2014 May 03;6(2):12.
Case report
Competing interests: None declared. Conflict of interests: None declared.
All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
Nature of Head & Neck
Tumours
Pilomatrix carcinoma of the head and neck: Case report and
literature review
CC Liu1, M Hoy1, TW Matthews1, K Guggisberg1, S Chandarana1*
Abstract
Introduction
Pilomatrix carcinoma is a rare
cutaneous malignancy of hair matrix
cells. They exhibit an aggressive and
infiltrative growth pattern and,
despite treatment to the local disease,
there is a high propensity for
recurrence. The literature reports a
poor prognosis associated with
regional spread occurring with distant
metastasis. The patient we report is
alive at over two years post-
operatively. With only one other case
of a head and neck pilomatrix
carcinoma ever reported in Canada,
our report and review of the literature
contributes to what little is known
currently about the treatment of this
malignancy.
Case report
In previously reported cases, regional
lymph node metastasis is always
associated with distant metastatic
disease on presentation, thus
rendering the treatment palliative in
nature. We present the unique case of
a 63-year-old man with a pilomatrix
carcinoma of the left parotid region.
He had bilateral cervical lymph node
spread but no detectable distant
metastasis. The patient underwent a
wide excision of the lesion with a
radical parotidectomy, temporal bone
resection, bilateral neck dissections,
and post-operative radiation.
Conclusion
The treatment of pilomatrix
carcinomas involve extensive surgical
management with wide margins. Post-
operative radiation in patients
without distant metastasis may
decrease the risk of local recurrence.
Introduction
Pilomatrix carcinoma is the rare
malignant variant of pilomatrixoma, a
cutaneous tumour of hair matrix cells
also known as the calcifying
epithelioma of Malherbe1. A literature
review using the Pubmed and Embase
databases yielded approximately 90
reported cases of pilomatrix
carcinomas. Fifty-one of these
occurred in the head and neck region.
Due to the paucity of head and neck
pilomatrix carcinoma cases, little is
known about the optimal
management, particularly in the case
of regional spread without distant
metastasis. In previously reported
cases, regional lymph node metastasis
is always associated with distant
metastatic disease on presentation,
precluding curative treatment. We
present the unique case of a 63-year-
old man with a pilomatrix carcinoma
of the left parotid region. He had
bilateral cervical lymph node spread
but no detectable distant metastasis.
The literature reports a poor
prognosis associated with regional
and concurrent distant metastasis.
The patient we report has done well
and is alive at over two years post-
operatively. There is only one other
case of a head and neck pilomatrix
carcinoma ever reported in Canada.
Our report and review of the
literature contributes to what little is
known currently about the treatment
of this malignancy.
Case report
A 63-year-old man presented to a
community Otolaryngologist with a
post-auricular mass of six months
duration. At the time of presentation,
it was ulcerated and draining. Aside
from a recent weight loss of 10
pounds, the patient was essentially
asymptomatic in terms of pain and
function. A CT scan showed a 3 cm
lesion which appeared to be arising
laterally in the left parotid gland, with
enlarged lymph nodes inferior and
deep to the gland. Biopsy revealed an
initial diagnosis of squamous cell
carcinoma.
The patient was subsequently
evaluated at the Tom Baker Cancer
Centre by the multidisciplinary head
and neck team and further
investigations were arranged. On exam,
there was a large, fixed mass in the left
posterior neck, measuring
approximately 6 cm in diameter (Figure
1).
Pathology review of the biopsy at a
tertiary centre resulted in a revised
diagnosis of pilomatrix carcinoma.
Additional imaging was undertaken to
evaluate the extent of disease. Both the
CT and MRI scans showed a large, 5 cm
x 3 cm mass in the left suprahyoid neck
region with involvement of the
overlying skin and bilateral cervical
lymphadenopathy.
The tumour extended to the major
vessels of the neck, abutting branches
of the left external carotid artery as
well as the internal jugular vein. There
was also gross invasion of the
sternocleidomastoid muscle at the
mastoid origin of the parotid gland. A
CT/PET scan showed bilateral cervical
lymph node disease but no evidence of
distant metastasis.
The patient underwent an excision of
the mass with 1.5-2 cm cutaneous
margins, along with a left extended
neck dissection, left radical
parotidectomy, left lateral temporal
bone resection and right modified neck
dissection (levels I-IV). He was treated
with adjuvant radiotherapy, receiving
4620 cGy of the 6600 cGy that was
prescribed because of patient non-
compliance. The patient did not attend
scheduled regular post-treatment
follow-up. Eighteen months later, he
presented with an isolated 1.6 cm
lymph node in his right supraclavicular
fossa. CT imaging of the neck, chest, and
*Corresponding author
Email: shamir.chandarana@ucalgary.ca
1 University of Calgary, Calgary, Canada.
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Licensee OAPL (UK) 2014. Creative Commons Attribution License (CC-BY)
FOR CITATION PURPOSES
: Liu CC, Hoy M, Matthews TW, Guggisberg K, Chandarana S. Pilomatrix carcinoma of the
head and neck: Case report and literature review. Head Neck Oncol 2014 May 03;6(2):12.
Case report
Competing interests: None declared. Conflict of interests: None declared.
All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
abdomen failed to demonstrate other
local, regional, or distant disease. Fine
needle aspiration of the mass
suggested squamous cell carcinoma.
Wide local excision of the mass was
recommended but the patient
declined.
Pathological Findings
On gross examination, the tumour
measured 5.8 x 4.2 cm and was found
to invade the left parotid gland and
surrounding skeletal muscle. There
was no extension into the temporal
bone. The left neck dissection yielded
five positive lymph nodes while there
were thirteen nodes positive for
metastasis on the right side.
The tumour showed a nodular
basosquamous proliferation with
areas of necrosis (Figure 2). The
advancing tumoural front showed
individual cells percolating through a
desmoplastic stroma. Tumoural nests
composed of pleomorphic basaloid
cells were sharply demarcated from
central keratinizing areas. Numerous
cells were captured in mitoses. Both
lymphovascular and perineural
invasion were present.
Discussion
Pilomatrix carcinomas (PC) are rare
malignant tumours of the hair matrix.
There have only been approximately
90 reported cases of pilomatrix
carcinomas, half of which occurred in
the head and neck region. This family
of matrical tumours also includes
pilomatrixomas and proliferating
pilomatrixomas. It is worthwhile to
distinguish these three entities both
clinically and histologically (Table 1).
Pilomatrixomas have a bimodal
distribution for age of onset with up to
60% diagnosed before the age of
202,3,4,5,6 and a later peak in the 60s-
70s5,6,7.
There is a female predominance
with a male: female ratio of 2:32,3,5,6.
Pilomatrixomas are typically smaller
in size (<2-3 cm), slow-growing, and
are regular in appearance8,9,10,11.
Recurrences are unlikely (3%) with
simple excision12. Histologically,
pilomatrixomas are characterized by
collections of uniform basaloid cells
with shadow cells and the absence of
local invasion, central necrosis, and
atypical mitosis.
Proliferating pilomatrixomas also
have a female predominance but have
been mostly reported in older patients
from 60-80 years old7,11. They can
grow to upwards of 5.5 cm but are
similar to ordinary pilomatrixomas,
typically symmetrical in appearance7,
11. Because proliferating pilomatri-
xomas are infiltrative in their growth
pattern, there is a greater likelihood for
recurrence after a simple excision
(14%)11.
Histologically, there is greater atypia
and a higher mitotic rate than is seen
with a pilomatrixoma2. Tissue invasion
and central necrosis are present. A
pilomatrix carcinoma has similar
characteristics to a proliferating
pilomatrixoma but with significantly
greater pleomorphism as well as the
presence of perineural and vascular
Figure 1: Ulcerated mass measuring 5.8 x 4.2 cm in the left parotid region.
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Licensee OAPL (UK) 2014. Creative Commons Attribution License (CC-BY)
FOR CITATION PURPOSES
: Liu CC, Hoy M, Matthews TW, Guggisberg K, Chandarana S. Pilomatrix carcinoma of the
head and neck: Case report and literature review. Head Neck Oncol 2014 May 03;6(2):12.
Case report
Competing interests: None declared. Conflict of interests: None declared.
All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
invasion3. Pilomatrix carcinomas
occur mostly in middle-aged patients,
with an average age of 45 to 60 years
old3,11,13,14,15,16,17,18. There is a strong
male predominance with a
male:female ratio of 3-5:13,11,13,14,16,19.
They can present as slow-growing
and asymptomatic nodules, rapidly
enlarging new nodules, or new rapid
growth in a long-standing nodule.
There is great variability in size, with
the largest tumour reported
measuring 20 cm9,20. The head and
neck region is most often affected and
the carcinoma may arise de novo or as
a result of malignant transformation
of a pre-existing pilomatrixoma18,21.
While it is a low-grade tumour, PCs
exhibit an infiltrative growth pattern
and are locally aggressive3,11. With
inadequate surgical management,
there is a great propensity for
recurrence, with recurrence rates
from 46-60%11,13,20. In our review of
51 cases of PCs occurring in the head
and neck (Table 2), 29 patients
underwent wide excisions for their
primary tumours with margins from
0.5-3cm. Twenty-three percent of
these patients experienced disease
recurrence. Twelve patients
underwent simple excisions as initial
treatment with a recurrence rate of
50%. Therefore, the consensus in
literature regarding surgical
treatment is wide excision with a
minimum margin of 5
mm3,5,10,11,13,14,15,16,19,21,22,23.
The potentially aggressive nature of
PCs has been demonstrated in cases of
extensive local invasion into
surrounding soft tissues and bony
structures, at either initial
presentation or at the time of
recurrence5,17,18,24,25.
Treatment involves wide excision of
the tumour along with affected bone
and soft tissues. Outcomes are good
with no evidence of disease at the
time of follow-up in the reported
cases17,18,24.
Distant metastasis has also been
reported6,13,20,26. The most common
sites of metastasis are the lungs, spine,
and abdominal viscera. Chemotherapy
and radiation was ineffective in
halting disease progression13,26,
suggesting that such treatments are
largely futile in cases of distant
metastasis. However, there may be a
role for adjuvant radiotherapy after
surgical excision (both wide and
simple), which has been used
effectively for local disease
control25,27,28,29.
Conclusion
In concordance with previous reports,
our case demonstrates the capacity
for PCs to invade locally and spread
regionally. In terms of treatment, our
case supports the approach of
aggressive and extensive surgical
management of the tumour with wide
margins. Due to the few number of PC
cases, there is little experience in using
radiation and chemotherapy as part of
the treatment for this malignancy.
Reports to date have shown very little
effect in using these therapies in
patients with distant metastasis.
However, there is some evidence to
suggest that radiation may be useful in
controlling local disease. Our case can
be added to the small body of literature
supporting the use of aggressive
surgery and adjuvant radiation in
patients without distant disease to
prevent local recurrence.
Consent
Written informed consent was obtained
from the patient for publication of this
Case report and any accompanying
images. A copy of the written consent is
available for review by the Editor-in-
Chief of this journal.
Abbreviations list
PC pilomatrix carcinoma
Authors’ Contribution
CCL participated in the literature
search and drafting, revising, and
approving the final manuscript. MH
Table 1: Clinical and histological characteristics of pilomatrixoma, proliferating pilomatrixoma, and pilomatrix
carcinoma.
Pilomatrixoma
Proliferating Pilomatrixoma
Pilomatrix carcinoma
Age
Bimodal distribution
- <20 yo2-5
- 60-70 yo4,7
Mostly elderly >60-707,11
Middle aged 45-60 yo3,11,13-18
Gender
M:F ratio 2:3 2-3,5,6
M < F 11
M:F ratio 3-4:1 3,13-14,16,19
Clinical
characteristics
- Single, slow growing, and
asymptomatic nodule9
- Cystic, symmetric11
- Recurrence rate 3%12
- Solid, symmetric11
- Locally invasive
- No metastatic potential
- Recurrence rate 14%11
- Irregular11
- Growth may be slow or rapid3
- Locally invasive3,10-11,25-26,30
- Can metastasize to lungs, bone,
abdominal viscera4
- Recurrence rate 46-60%11,13,20
Histology
- Collections of basaloid cells,
usually at the periphery11
- Cells are uniform, few mitotic
figures23
- Shadow cells present2
- No central necrosis2
- No lymphovascular or
perineural invasion2
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Licensee OAPL (UK) 2014. Creative Commons Attribution License (CC-BY)
FOR CITATION PURPOSES
: Liu CC, Hoy M, Matthews TW, Guggisberg K, Chandarana S. Pilomatrix carcinoma of the
head and neck: Case report and literature review. Head Neck Oncol 2014 May 03;6(2):12.
Case report
Competing interests: None declared. Conflict of interests: None declared.
All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
participated in revising and approving
the final manuscript.
TWM participated in revising and
approving the final manuscript. KG
participated in drafting the pathology
section of the manuscript, and
revising and approving the final
manuscript. SC participated in
revising and approving the final
manuscript.
References
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Location of
tumor
Number
of Cases
Treatment (number
of cases)
Follow up (number of
cases)
Posterior neck3,9,
18,20,21,25,26
11
Wide excision (5)
NED (4), DOD (1)
Simple excision (4)
NED (2), DII (1), LFU (1)
Excision, MNS (2)
NED (1), AWD (1)
Lateral/anterior
neck3,28,33
3
Wide excision (2)
NED (1), LFU (1)
Excision, MNS (1)
LFU
Scalp3,5,11,13,15,16,
22,29,33
10
Wide excision (7)
NED (4), LFU (3)
Simple excision (3)
NED (1), DOD (1), LFU (1)
Eye region3,4,14,
23,32,33
6
Wide excision (3)
NED (1), LFU (2)
Simple excision (3)
NED (2), LFU (1)
Cheek6,11,27,30,33
6
Wide excision (4)
NED (2), LFU (2)
Simple excision (1)
NED
Excision, MNS (1)
LFU
Pre-auricular3,11,
33,34
5
Wide excision (3)
NED (2), DOD (1)
Simple excision (1)
LFU
Excision, MNS (1)
NED
Post-auricular11,
17,19
3
Wide excision (2)
NED (2)
Simple excision (1)
NED
Ear33
2
Wide excision (2)
NED (1), LFU (1)
Nose, lips,
forehead10,33
3
Wide excision (1)
NED
Incomplete excision
(2)
DII (1), LFU (1)
Scapula,
supraclavicular
24,31
2
Wide excision (1)
NED
Excision, MNS (1)
LFU
MNS - margin not specified, DOD - died of disease, AWD - alive with disease
NED - no evidence of disease, DII - died of intercurrent illness, LFU - lost to follow-
up
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FOR CITATION PURPOSES
: Liu CC, Hoy M, Matthews TW, Guggisberg K, Chandarana S. Pilomatrix carcinoma of the
head and neck: Case report and literature review. Head Neck Oncol 2014 May 03;6(2):12.
Case report
Competing interests: None declared. Conflict of interests: None declared.
All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
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... Malignant pilomatrixoma, in other words calcifying epitheliocarcinoma of Malherbe was first described by Lopansri and Mihm in 1980. [1][2][3][4][5] It is an extremely rare malignant hair follicle carcinoma. 2,4,[6][7][8][9] Until now there were just 136 cases reported in the English literature. ...
... [1][2][3][4][5] It is an extremely rare malignant hair follicle carcinoma. 2,4,[6][7][8][9] Until now there were just 136 cases reported in the English literature. 9 The tumour is locally aggressive, has a strong tendency to reoccur and can metastasize to lungs, spine, abdominal viscera, regional lymph nodes and brains. ...
... 9 The tumour is locally aggressive, has a strong tendency to reoccur and can metastasize to lungs, spine, abdominal viscera, regional lymph nodes and brains. 1,2,4,[6][7][8][9] The most common sites of tumour presentation being head and neck nevertheless it can also present on the back, upper and lower extremity, chest inguinal and axillary regions and on the buttocks. 1,5,6,8,9 This is the first known case of malignant pilomatrixoma in our department. ...
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p class="abstract">Malignant pilomatrixoma is a rare malignancy with just 136 cases reported in the English literature. The tumour is described as locally aggressive, with possibility of reoccurrence and distal metastasis. Histology remains the gold standard in diagnostic. Wide surgical excision is preferred to simple excision. In the present study a case of first malignant pilomatrixoma in our hospital is described. We made an excision of the tumour and the tissue was sent on histopathology. The skin defect was covered with a local ulnar flap. Through our case report and literature review we focus on the most suitable treatment option. The tumour was not excised with appropriate safety surgical margin based on the histopathology report. Patient refused another excision and any additional treatments. The most optimal treatment has not yet been established. Most of the article’s state that primary wide surgical excision should be done, with surgical margins still being debateable. The best results were given when using Mohs micrographic surgery.</p
... 24 It is necessary to do a wide excision of this tumor with a 5mm clearance margin to prevent recurrence which is seen in 14% of cases and also progression to pilomatrix carcinoma which till now 90 cases are reported in the literature, half of which were in head and neck region. 25 Sometimes pigmented nodular lesions in the scalp can be mistaken for EC as pigmentation may not be evident in dark-skinned people. We encountered two such cases in our study. ...
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Background: Cystic lesions of the scalp with a clinical diagnosis of an epidermoid cyst are encountered in clinical practice. However, these lesions had a different diagnostic interpretation on histopathology examination. Therefore our study focused on the lesions clinically resembling the presentation of epidermoid cyst in the scalp region. Materials and Methods: Forty-one cases of scalp lesions with a clinical diagnosis of epidermoid cyst over 2 years were reviewed. Details of the patients such as clinical diagnosis, age, gender, duration, gross findings, and histopathology diagnosis were obtained from the medical and histopathology records. Results: Epidermoid cysts diagnosed both clinically and on histopathology examination accounted for 29.26% whereas the clinical mimickers constituted 70.73%. The mean age of the patient was 35-years with an equal sex ratio. The commonest location was the parietal region (41.37%). Benign and malignant lesions consisted of 97.56% and 2.4% respectively. A trichilemmal cyst was the most common clinical mimicker diagnosed. Conclusions: Clinicians should be aware of wide differential diagnosis of epidermoid cyst in the scalp region as the management and outcome vary with each lesion. Histopathology examination proved to be a diagnostic tool in differentiating these lesions.
... Though the majority of pilomatrical carcinomas develop de novo, malignant transformation from a preexisting pilomatrixoma has been reported [6,7]. Pilomatrical carcinomas mostly occur in the head and neck, upper extremities and buttocks [1][2][3][4][5][6][7][8]. Although, it was considered to be a low-grade malignant tumor, its significant metastatic potential is being evident [1,4,5,7]. ...
... Malignant pilomatrixomas or pilomatrix carcinoma was fi rst described in 1980 by Lopansiri and Mihm and till date around 80 cases have been reported [1]. Most of these tumours were located in head and neck region followed in sequence by upper limbs, lower limbs and scalp [2]. A male preponderance (5:1) has been seen and these tumours are more commoner in age >50 years and white race [3,4]. ...
... Some physicians consider a clear surgical margin of 2 cm as adequate.7 In a review of 51 cases of PC in the head and neck byLiu et al, 29 patients who underwent wide excisions of their primary tumors with margins between 0.5 cm and 3 cm had a recurrence rate of 23%.8 In contrast, 12 patients who underwent simple excisions as initial treatment had a recurrence rate of 50%. ...
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Pilomatrix carcinoma is a very rare malignant tumor. The present article raises awareness of the clinical features, diagnosis, and treatment of this malignancy. The clinical data of a case of pilomatrix carcinoma of the left temporal region treated at the First Affiliated Hospital of Nan Chang University, Nan Chang, China, in April 2017 were analyzed retrospectively, and the related literature was reviewed. The patient underwent a wide excision of the mass, and microscopic examination revealed that the tumor showed the typical features of pilomatrix carcinoma. Pilomatrix carcinoma is a very rare tumor with approximately 130 cases reported in the English literature. An optimal treatment regimen has not been established; however, wide surgical excision is recommended to avoid recurrence when the staging shows no metastasis, and adjuvant radiotherapy might be beneficial for local tumor control. Pilomatrix carcinoma is considered to be a low-grade malignant tumor and shows an aggressive growth pattern. A wide surgical excision and radiotherapy are the main modalities of treatment.
... Malignant pilomatrixomas or pilomatrix carcinoma was fi rst described in 1980 by Lopansiri and Mihm and till date around 80 cases have been reported [1]. Most of these tumours were located in head and neck region followed in sequence by upper limbs, lower limbs and scalp [2]. A male preponderance (5:1) has been seen and these tumours are more commoner in age >50 years and white race [3,4]. ...
Article
Pilomatrix carcinoma is extremely rare malignant tumours arising from hair follicles with around 80 cases reported till date. Owing to the paucity of literature and similarity of clinic pathological features with its benign counterpart they are oft en misdiagnosed. These tumors are known to be locally aggressive with low albeit significant metastatic potential. Surgical excision is the mainstay of treatment with adjuvant radiotherapy being used historically for improving local control. We present a case of an extremely unusual site (breast) of pilomatrix carcinoma. To the best of our knowledge no case of pilomatrix carcinoma of the mammary skin has been reported till date.
... The majority of pilomatrical carcinomas develop de novo; however, the malignant transformation from a pre-existing pilomatricoma has been reported [6]. Pilomatrical carcinomas mostly occur in the head and neck [7], upper extremities, and buttocks. Rare tumors have been reported in the axilla and inguinal regions [8]. ...
Article
Pilomatrixoma was first described in 1880. Since then, approximately 73 carcinomas have been reported in the literature. A 57year-old man was evaluated for a solitary nodule on the left temporoparietal scalp. Biopsy revealed pilomatrixoma. The lesion was treated by wide surgical resection. The patient was free of disease 6months after surgery. We report another case of pilomatrix carcinoma arising from the scalp.
Article
Pilomatrix carcinoma is a rare, low grade malignant neoplasm arising from the hair matrix. This neoplasm can exhibit local aggressive behaviour and occasionally distant metastasis. In this report, we present a case of malignant pilomatricoma in the eyelid. Differential diagnosis and treatment are discussed.
Article
Pilomatrix carcinoma is a rare malignant counterpart of pilomatricoma. To our knowledge, only approximately 90 cases have been published in English literature. Pilomatrix carcinoma is locally aggressive and occasionally shows rapid progression infiltrating to the muscle, bone and vessels. We report a case of pilomatrix carcinoma that developed in a 38-year-old man and started to grow after a long stable period, relapsed for a short time and infiltrated into the muscle underneath. While the initial skin biopsy showed histopathological findings consistent with pilomatricoma, the recurrent tumor contained marked cellular atypia and an aggressive growth pattern. Although it is still controversial whether pilomatrix carcinoma arises de novo or through malignant transformation of a pilomatricoma, the present case might be caused by the latter process considering the patient's clinical course. beta-catenin is a downstream effecter in the canonical pathway of Wnt, acting as a signal for cell differentiation and proliferation. The characteristic nuclear staining pattern of beta-catenin in the basaloid tumor cells, which is usually observed in pilomatrix carcinoma, supported the diagnosis of pilomatrix carcinoma in the present case.
Article
We report the case of a 41-year-old mentally retarded male with recurrent pilomatrix carcinoma of the occipital region which invaded the occipital bone, left cerebellum and left temporal lobe. At his initial presentation the patient had a craniotomy and subtotal excision of the lesion with positive margins. He received no adjuvant therapy. After an early intracranial recurrence he had subtotal debulking and was referred for external beam radiotherapy. At 27 months follow-up after adjuvant external beam radiotherapy the intracranial component has not progressed and the patient remains clinically well.
Article
Pilomatrix carcinoma, the malignant equivalent of pilomatrixoma, is rare among skin cancers. In the literature, there have been 80 cases of pilomatrix carcinoma reported, and among them nine were with metastases. The clinical presentation of this case is suggestive for the biology and of the usual history of this neoplasm. The patient was a 53-year-old male who had been treated 2 years earlier for a pilomatrix carcinoma located in the posterior part of the neck. The clinical presentation had been characterised by sudden paraplegia caused by vertebral collapse at T4 due to bone metastases. The patient underwent a first surgery for vertebral stabilisation and medullary decompression; then, he had a second operation for the resection of the local relapse of the tumour. Literature review and analysis of this case show that the pilomatrix carcinoma should be regarded as a highly locally aggressive tumour, with a high rate of local recurrence as well as metastases.
Article
A matrical carcinoma (pilomatrix carcinoma) of the scalp is described--the first reported case in this site. This malignant hair follicle tumour must be distinguished histologically from benign pilomatricoma and proliferating trichilemmal tumour, which can have similar features.
Article
Pilomatrixoma, a neoplasm of hair germ matrix origin, is one of the most common cutaneous appendage tumors in patients 20 years of age or younger. Our review of cutaneous adnexal tumors in the first 2 decades of life showed that pilomatric lesions accounted for approximately 75% of cases. We examined 76 tumors from 69 patients between the ages of 8 months and 19 years (average age, 8 years). The female-to-male ratio was 3:1. We noted a predilection for the head and neck region, as has also been reported by other investigators. Sixty-nine tumors were examples of typical or classic pilomatrixoma; four of them showed the unusual histologic feature of transepidermal elimination or perforation. None of the tumors in the typical category recurred during a follow-up period that averaged 8.7 years. Seven of the lesions with atypical histologic features were examples of aggressive pilomatrixoma (three cases) and pilomatrical carcinoma (four cases). The average age of these seven patients was similar to that of the overall group. The pilomatrical carcinomas were characterized by invasive nests of tumor cells with irregular borders, large vesicular nuclei, prominent nucleoli, multiple mitotic figures, and focal necrosis. A desmoplastic stroma surrounded the infiltrating nests of tumor. Two of the four pilomatrical carcinomas recurred 2 and 6 months, respectively, after initial excision. The aggressive pilomatrixomas had a similar infiltrative growth pattern, but they did not exhibit the same degree of worrisome cytologic features. Single cell necrosis, identifiable mitotic figures, and prominent nucleoli differentiated the atypical from the typical pilomatrixomas. One of the three aggressive pilomatrixomas recurred after 2 months.(ABSTRACT TRUNCATED AT 250 WORDS)
Article
We report a case of giant pilomatrix carcinoma and review the literature on this rare tumor.
Article
Pilomatrix carcinomas are rare neoplasms of the skin that may be locally aggressive or metastatic. The differentiation of these tumors from benign pilomatrixomas depends on a constellation of microscopic features, some of which may be equivocal or absent in individual biopsy specimens. We encountered a tumor with distinct pilomatrix differentiation (lobulated nests of basaloid cells, ghost cells, focal calcification) that recurred multiple times and ultimately invaded the cranial vault. Despite this aggressive behavior, the tumor was difficult to separate from benign pilomatrixoma on morphologic grounds. Because DNA content flow cytometry has proved useful in the prediction of aggressive behavior in various solid tumors, we analyzed this neoplasm by flow cytometry. Neither aneuploid peaks nor a high proliferative fraction were seen in this example of pilomatrix carcinoma.