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OJHAS Vol. 10, Issue 2:
(Apr-Jun 2011) |
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| Myxoid
Neurothekeoma of the Nipple |
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Thejaswini
MU, Vijaya Shankar, Indira, Swarna Shivakumar,
Department
of Pathology, Adichunchanagiri Institute Of Medical Sciences, BG Nagara,
Karnataka, India. |
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Address for Correspondence |
Assistant Professor,
Department of Pathology,
ESIPGIMSR, Rajajinagar,
Bangalore - 560010,
Karnataka, India.
E-mail:
thejaswinimu@gmail.com |
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Thejaswini
MU, Vijaya Shankar, Indira, Shivakumar S. Myxoid
Neurothekeoma of the Nipple. Online J Health Allied Scs.
2011;10(2):26 |
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Submitted: June 2,
2011; Accepted: Jul 16, 2011; Published: Jul 30, 2011 |
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| Abstract: |
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Neurothekeomas
are rare benign cutaneous neoplasms of nerve sheath origin. They are
primarily found in the superficial soft tissue and are also known as
dermal nerve sheath myxomas. They are commonly found on the upper extremities,
head and neck followed by trunk. Here is an unusual presentation of
neurothekeoma occurring as a polypoidal lesion of the nipple in a young
female patient.
Key Words:
Neurothekeoma;
Dermal nerve sheath myxoma
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Neurothekeomas
or dermal nerve sheath myxomas are slow growing tumors of nerve sheath
origin. They are common on the upper extremities, head and neck. We
are reporting an unusual presentation of neurothekeoma as a polypoidal
mass over the nipple, clinically mimicking a nipple papilloma.
A 26 years old
south Indian woman presented with a slow growing, non-tender, polypoidal
lesion over the left nipple for 3 years. There was no associated nipple
discharge or lump in the breast. A clinical diagnosis of nipple papilloma
was made; the lesion was excised completely and sent for histopathological
examination.
The excised
specimen was a skin covered polypoidal tissue, measuring 2 cm in greatest
dimension with a stalk measuring 0.5 cm; the cut surface was grey-white,
lobulated and glistening (Fig 1). Microscopic examination revealed a
lobular, hypocellular neoplasm in the dermis (Fig 2). Bland spindle
shaped tumor cells with interspersed collagen bundles were seen embedded
in a myxoid stroma (Fig 3). Nuclear pleomorphism and mitotic figures
were absent. Immunohistochemistry revealed strong S-100 positivity of
the tumor cells (Fig 4). EMA, CD34 and HMB-45 were negative. The surgical
margins were completely free of tumor. With the above features, a diagnosis
of myxoid neurothekeoma was given.
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Figure 1: Photograph showing
skin covered polypoidal tissue with a grey white glistening surface |
Figure 2: Photomicrograph
showing a lobular, hypocellular neoplasm in the dermis (x100) |
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Figure 3: Photomicrograph
showing abundant myxoid stroma containing spindle shaped tumor cells
(x100) |
Figure 4: Photomicrograph
showing strong S-100 positivity of the tumor cells (x100) |
In the year
1969, Harkin and Reed first described a rare neoplasm arising in the
endoneurium of peripheral nerves, characterized by abundant myxoid matrix
and called it myxoma of nerve sheath.(1) The term neurothekeoma was
coined by Gallager and Helwig who first published a large series of
this tumor in 1980. Neurothekeoma was described in detail by Pulitzer
and Reed in 1985.(1) Females in the 2nd and 3rd
decades of their lives were more commonly affected with rare occurrence
in infants and elderly. These tumors showed prediction to head and neck,
arms, shoulders and trunk; other uncommon sites of involvement were
subungal region, eye and spine. Out of the 300 cases of neurothekeoma
described by Pulitzer and Reed and Gallager and Helwig, only one case
had the lesion over the breast. Ours is the first reported case of neurothekeoma
arising over nipple and presenting as a polypoidal growth.
In
1986, Rosati described a similar tumor with high Cellularity and named
it cellular neurothekeoma. Fetsch and others reported that cellular
neurothekeoma exhibited different immunohistochemical profile and occurred
in younger patients with a predilection to head when compared to myxoid
neurothekeomas. 3 distinct types of neurothekeoma were described (2):
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Hypocellular
type (myxoid): The hypo cellular group consisted of well circumscribed
lobular tumors with prominent myxoid stroma and positive for S-100 and
collagen type IV and variably positive for EMA.
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Cellular
type: The cellular groups were composed of ill defined nests and fascicles
of spindle cells with scant mucin and S-100 negativity. The cellular
neurothekeomas do not possess any evidence of neural differentiation
and therefore represented cutaneous neoplasm of undetermined cellular
origin.
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Mixed type:
Variable Cellularity and mucin content with poor demarcation and variable
results with immunomarkers.
However, Colonje
et al showed that the cellular neurothekeoma was negative to PGP 9.5
and strongly positive for NK/C3. Thus they proposed that cellular neurothekeoma
represented epitheloid variant of pilar leiomyoma.(3)
Laskin
et al in 2000 showed that myxoid/ hypocellular variety occurred more
commonly in male patients with a peak incidence in fourth decade and
were found in both upper and lower limbs and back.(4) This was in contrast
to cellular neurothekeoma which affected more female patients with peak
incidence in second decade of life in upper body distribution.
The
fact that tumor location changes with age was shown by Papadopoulos
et al. In children, head and neck comprised 45.5% of cases as compared
to 24.4% in adults. In adults, upper extremity tumors were more common.(5)
Hornick et al in their study of 133 cellular neurothekeomas, showed
that 35% arose in upper extremities, 33% in head and neck, 17% in lower
limb and 15% on trunk; of all these cases, face and shoulder were the
most commonly affected sites.(6)
The
tumors range from 0.4 to 4.5 cm in greatest dimension, with a rubbery
to firm consistency, and on cut section, small, well-demarcated, translucent
or whitish (rarely yellowish), glistening, mucoid nodules are often
noted.(7)
In
our patient, histology showed a well circumscribed, multilobulated tumor
in the dermis, composed of bland stellate and spindle cells dispersed
in abundant myxoid stroma. Further, immunohistochemistry demonstrated
S-100 positivity of the tumor cells, confirming the neural origin of
this tumor.
The
tumors are treated by wide local excision and surgical clearance as
local invasion and tumor recurrence rate is high.(8)
To our knowledge,
this is the first case report of a myxoid neurothekeoma involving the
nipple and presenting clinically as a Polypoidal mass.
- Connolly
M, Hickey JR, Intzedy L, Pawade J and de Berker DAR. Subungal neurothekeoma.
J Am Acad Dermatol. 2005;52(1):159-162.
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Fetsch JF,
Laskin WB, Hallman JR, Lerpton GP, Miettinen. Neutrothekeoma – An
analysis of 178 tumors with detailed immunohistochemistry data and long
term patient follow up information. Am J Surg Pathol. 2007;31(7):1103-1114.
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Calonje
E, Wilson-Jones E, Smith NP, et al. Cellular 'neurothekeoma':an epithelioid
variant of pilar leiomyoma? Morphological and immunohistochemical analysis
of a series. Histopathology. 1992;20:397-404.
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Laskin WB,
Fetsch JF, MiettinemM. The "neurothekeoma":immunhistochemical
analysis distinguishes the true nerve sheath myxoma fromits mimics. Hum Pathol.
2000;31:1230-1241.
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Papadopoulos
EJ, Cohen PR, Hebert AA. Neurothekeoma – report of a case in an infant
and review of the literature. J Am Acad Dermatol. 2004;50(1):129-134.
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Hornick
JL, Fletcher CDM. Cellular neurothekeoma: Detailed characterization in
a series of 133 cases. Am J Surg Pathol. 2007;31(3):329-340.
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Argenyi
ZB, LeBoit PE, Santa Cruz D, Swanson PE, Kutzner H. Nerve sheath myxoma (neurothekeoma) of skin: light microscopic and immunohistochemical
reappraisal of the cellular variant. J Cutan Pathol 1993;20:294-303.
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Ward JL, Prieto
VG, Joseph A, Chevray P, Kronowitz S, Sturgis EM.Neurothekeoma. Otolarynol
Head Neck Surg 2005;132:86-89.
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