ARTICLE
Multiple skin carcinomas are not uncommon and can be observed in patients
with genetic defects, such as Gorlin syndrome or xeroderma pigmentosum,
in patients who were exposed to arsenic, photochemotherapy, hydrocarbons
or X ray therapy, in immunosuppressed patients [1] or for unknown reasons.
We report the case of a 75-year old woman who successively developed a
porocarcinoma, multiple squamous cell carcinomas and a tricholemmal carcinoma
within a one-year period.
Case report
A 75-year old woman was first seen for a tumor located on her left leg.
She had had a lesion for more than 4 months, that was first treated by
various topical treatments, including neomycin. She developed an intense
contact dermatitis of the left leg centered by an ulcerated tumor of 2.5
cm diameter. Clinical examination revealed multiple other tumors: a bluish
nodule that seemed cystic and a keratotic tumor of the right leg, two
keratotic papules of 1 cm diameter on her arm and thigh, and multiple
actinic keratoses on her forehead.
Histopathological examination of the three small tumors showed typical
Bowen's disease in two samples (thigh and arm) and a well differentiated
squamous cell carcinoma (right leg). The lesion of the right leg was an
intradermal epithelial tumor made of large pseudo-cystic structures (Fig.
1). The cavities were bordered by aggregations of round cells
that showed crowded and atypical large nuclei with numerous mitotic figures
(Fig. 2). Both basaloid
and squamous cells were identified as well as dyskeratotic cells which
allowed the diagnosis of pseudo-cystic squamous cell carcinoma. Staining
for carninoembryonic antigen was negative.
The ulcerated tumor of the left leg invaded
the whole dermis and hypodermis, and was made of nests and strands of
small and regular basophilic poral cells. The tumor was sharply demarcated
from the normal epidermis at each side (Fig.
3). Numerous atypical nuclei and mitoses were present. In the
deep part of the dermis small nests of cells detached from the central
tumor and were surrounded by clefts that made a sharp demarcation between
the tumoral cells and the stroma. Immunohistochemical staining for carcinoembryonic
antigen proved positive in the malignant cells, showing the presence of
some lumina inside the deep nests of cells. All these features were those
of trabecular eccrine porocarcinoma, without remnants of previous benign
eccrine poroma. Because the tumor was incompletely removed, a surgical
excision down to the underlying fascia was necessary. The same masses
of malignant poral cells were observed in the dermis and sucutis. Six
months after surgery, ulcerated nodules appeared in the center of the
cicatricial area of the leg (Fig.
4). Histopathological examination revealed a recurrence of the
eccrine porocarcinoma with intra-lymphatic nests of basophilic cells in
the deep part of dermis on both sides of the tumor. A second large and
deep excision was then performed. There were no lymph node enlargement
or visceral metastases. This tumor did not recur during a 3 year-follow-up.
Nevertheless, one year after the first surgery, the patient developed
a large exophytic and ulcerated tumor of the forehead (Fig.
5) which showed a very rapid growth over 3 months, reaching 2.5
cm in diameter.
Histopathological examination showed large masses of clear atypical
cells (Fig. 6) with a
trabecular growth pattern and invasion of the deep dermis. Typical features
of outer root sheath differentiation were found, especially palisading
of clear cells with subnuclear vacuolization in the periphery of the tumor
lobules. Areas of tricholemmal keratinization were noted in some of the
tumoral strands. The nuclei were very large and the cell cytoplasm was
strongly PAS-positive. Multiple mitotic figures were observed, most of
them being atypical. All these features were typical of tricholemmal carcinoma.
During a 2-year follow-up after excision, there was no recurrence of this
facial tumor. This patient was carefully questioned about her past medical
history. She had neither past visceral neoplasms nor lymphoma or leukaemia.
She had never received immunosuppressive treatment, X-ray therapy or exposure
to arsenic. There was no familial history of cancer. This patient was
a native French woman and had not been overexposed to solar irradiation
for occupational reasons, although she presented with numerous actinic
keratoses.
Discussion
To our knowledge, this is the first report of a single patient presenting
in a one-year period of time multiple carcinomas of unrelated histogenesis.
Both eccrine porocarcinoma [2, 3] and tricholemmal carcinoma [4, 5] are
very unusual neoplasms. Eccrine porocarcinoma of the trabecular type [3]
is often very invasive and recurrences are frequent. Despite local recurrence,
our patient did not develop metastatic disease, which seems to be more
frequent in the epidermotropic type of this neoplasm [3, 6]. The main
histopathological features that are important for the diagnosis were the
trabecular pattern, intracytoplasmic ducts, poral basophilic cells with
atypical nuclei and abnormal mitoses and clefts around the tumoral cells.
The tumor of the forehead was also very characteristic of a tricholemmal
carcinoma [4, 5]. This neoplasm occurs in elderly patients, shows a rapid
course and usually affects the face. In our patient, tumoral cells showed
the very typical features of outer root sheath cells. Tricholemmal carcinoma
has certainly malignant histological features, but recurrences or metastases
are uncommon [4, 5].
The diagnosis of cystic squamous cell carcinoma can be difficult. As
stated by Ackerman et al. [7], differentiating proliferating cystic
follicular neoplasm [8, 9] and squamous cell carcinoma is "a vexing problem".
The tumor illustrated in Figures
1 and 2 is very
similar to the squamous cell carcinoma demonstrated by Ackerman et
al. on p. 566 of their book [7]. It is likely that this neoplasm represents
a rare variant of pseudo-cystic and poorly differentiated squamous cell
carcinoma [7]. This tumor was obviously malignant by cytological criteria.
Eosinophilic cells and keratinization were both consistent with an unusual
form of squamous cell carcinoma. Because this tumor was not connected
with the overlying epidermis, other carcinomas could be discussed. The
diagnosis of sweat gland carcinoma is unlikely, because of the presence
of numerous eosinophilic cells with features of keratinization, absence
of ductal structure, and negative staining for carcinoembryonic antigen.
A pilar origin cannot be totally ruled out, but we could not find clear
cut evidence in favor of the hypothesis of pilar carcinoma.
The occurrence of multiple carcinomas in a single
patient could be fortuitous, but five unrelated malignant cutaneous tumors
were removed in a one-year period of time in this case. Multiple Bowen's
diseases are not uncommon, but we did not find any similar case associated
with adnexal and squamous cell carcinomas in the literature. Nevertheless,
we could not detect a predisposing factor in this patient who remained
in good general health during the follow-up. A genetic disease is unlikely,
since the patient did not develop skin neoplasms before the age of 75
years. Multiple adnexal tumors are described in the Muir and Torre syndrome,
but they are mainly of sebaceous origin. In this patient, no other internal
malignancies could be found in association with the skin findings, and
she is still alive more than 4 years after the first tumor occurred. External
carcinogenic factors are not known to induce adnexal neoplasms and none
of those factors previously described could be evidenced in our patient.
The occurrence of tricholemmal carcinoma in a 39-year-old organ transplant-recipient
has been reported [5]. Our patient had a normal lymphocyte count and had
never been treated by immuno- suppressive drugs. One of the cases of tricholemmal
carcinomas described by Swanson et al. was associated with past
multiple basal cell carcinomas [10]. Since the majority of tricholemma
carcinomas are located on sun-exposed areas, it can be hypothesized that
actinic damage could play a role in this type of tumor. Eccrine porocarcinoma
developed in a 12-year-old child with xeroderma pigmentosum and could
have therefore been enhanced by abnormal DNA repair mechanism [3]. It
is likely that carcinogenetic factors involved in the development of squamous
or basal cell carcinomas could also play a role in some
cases of adnexal carcinomas. Apart from sun exposure, no specific factor
could be evidenced in our patient.
REFERENCES
1. Bouwes Bavinck JN, Van Zuuren EJ, Ter Schegget J. Cutaneous warts
and carcinomas. In: Skin diseases after organ transplantation.
Euvrad S, Kanitakis J, Claudy A, eds. John Libbey Eurotext, Montrouge,
1998: 122-30.
2. Shaw M, McKee PH, Lowe D, Black MM. Malignant eccrine poromas: a
study of twenty-seven cases. Br J Dermatol 1982; 107: 675-81.
3. Poiares-Baptista A, Tellechea O, Reis JP, Cunha MF, Figueiredo P.
Porocarcinome eccrine. Revue de 24 cas. Ann Dermatol Venereol 1993;
120: 107-15.
4. Reis JP, Tellechea O, Cunha MF, Poiares Baptista A. Tricholemmal
carcinoma: review of 8 cases. J Cutan Pathol 1993; 20: 44-9.
5. Wong TY, Suster S. Tricholemmal carcinoma. A clinicopathologic study
of 13 cases. Am J Dermatopathol 1994; 16: 463-73.
6. Maeda T, Mori H, Matsuo T, Nakagawa JH, Yamauchi H, Tuziguchi H,
Hongou Y. Malignant eccrine poroma with multiple visceral metastases:
report of a case with autopsy findings. J Cutan Pathol 1996; 53:
566-70.
7. Ackerman AB, De Viragh PA, Chongchitnant N. Neoplasms with follicular
differentiation. Lea & Febiger, Philadelphia, 1993: 566-70.
8. Mehregan AH, Lee KC. Malignant proliferating tricholemmal tumors.
Report of three cases. J Dermatol Surg Oncol 1987; 13: 1339-42.
9. Weiss J, Heine M, Grimmel M, Jong EG. Malignant proliferating trichilemmal
cyst. J Am Acad Dermatol 1995; 32: 870-3.
10. Swanson PE, Marrogi AJ, Williams DJ, Cherwitz DL, Wick MR. Tricholemmal
carcinoma: clinicopathologic study of 10 cases. J Cutan Pathol
1992; 19: 100-9.
|
Printable version
