ARTICLE
Auteur(s) :, Franco
Picciotto1,*, Alessandro Zaccagna1,
Giovanni DeRosa1, Alberto Pisacane1, Paolo
Puiatti1, Enrico Colombo2, Fabrizio
Dardano2, Antonio Ottinetti2
1Operative Unit of Surgical Dermatology, Department
of Pathology, Institute for Cancer Research and Treatment (IRCC),
Strada Provinciale 142, Km 3.95, 10060 Candiolo, Italy Fax: (+39)
01 19 93 32 25.
2Department of Dermatology, Department of Pathology,
Ospedale S. Andrea, Vercelli
accepté le 20 Juin 2004
Clear cell sarcoma is a rare soft tissue tumor that occurs
predominantly in the extremities of young adults. Originally
described by Enzinger in 1965 [1] it constitutes 1% of all soft
tissue sarcoma [2]. It has also been referred to as “malignant
melanoma of soft parts” [1] because of its proposed neural crest
origin, the presence of melanin and positive melanoma associated
immunostaining with HMB-45, melanin synthesis, expression of S-100
protein and ultrastructural evidence of melanosomes [3]. This term
is now infrequently used because histopathological and
cytogenetical diagnostic tools have demonstrated that clear cell
sarcoma is a distinct clinicopathologic entity that behaves like a
high-grade soft tissue sarcoma. Prognosis is reported to be poor
due to the great propensity to form regional and distant
metastases. Local recurrence is common. Metastases occur in the
lung (59%), lymph nodes (53%) and bone (22%) [4]; less common sites
of metastases are the liver, brain and heart [4]. Lymph node
metastases occur in a high percentage of cases and symptomatic
lymph node involvement is a highly unfavourable factor, as reported
in the literature [4]. Early diagnosis and initial radical surgery
are essential for a favourable outcome. Once regional lymph node
metastases or hematogenous dissemination has occurred, the
prognosis is dismal.We report a case of clear cell sarcoma of the
hand in which lymph node biopsy was carried out prior to radical
surgery. This method may well be an efficient means to early
diagnosis of regional lymph node metastasis in this type of
neoplasm in the future.
Patient and methods
A 43-year-old male patient came under observation in August 2002,
in good general health. About one year previously he had noted a
slowly growing nodular lesion that had reached a size of about 1.5
× 1.5 cm located in the subcutaneous tissue of the palm of the left
hand at the 1st interdigital space.
Apart from slight pain on manual work, he reported no other
symptoms. There was no alteration in the cutaneous area overlying
the lesion, which was of normal colour and aspect. There were no
palpable lymph nodes in the superficial regions and in particular
in the left axilla. Considering the recent onset and evolution of
the lesion, we decided to proceed to an excisional biopsy.
Histological examination revealed a well defined non-encapsulated
multinodular lesion, situated in the dermis and subcutaneous
tissue, with no connection to the overlying epidermis. Nodules were
mainly constituted of fused cells with eosinophilic cytoplasm and
minor polygonal cells. Both cell types had vescicular nuclei with
prominent nucleoli (( figure 1 ), ( figure 2 )). The mitotic
activity was low. The immunophenotypic profile demonstrated a
diffuse positivity for S-100 protein and a focal but strong
positivity for HMB45 and NSE. Negative cytokeratin and CD117.
Clear cell sarcoma was diagnosed, based on histological and
immunohistochemical examination.
The patient was re-operated and treated with a wide local
incision that was repaired by skin graft taken from the left
subclavicular region. Before increasing the surgical scar, SLNB was
carried out with lymphoscintigraphy with 4 intradermic injections
near to the scar area, 22.2 Mbq of 99mTc-NANOCOLL and 4
subcutaneaous perilesional injection of blu-dye. Thus a lymph node
was detected in the left axilla that, examined according to the
method described by Cochran et al. [5], was micrometastase positive
for malignant neoplasia (( figure 3 ), ( figure 4 )). Madden [6, 7]
level I-II and III radical left axillary lymphadenectomy was
carried out after 12 days. Histology examination revealed no
metastasis in the 11 exported lymph nodes. Clinical follow-up and
total body CT scan carried out in March 2004 (20 months after the
primitive tumour excision), showed no local relapse or visceral
and/or lymphnode metastases.
Discussion
Clear cell sarcoma or malignant melanoma of soft tissue, is a rare
tumor accounting for a small percentage of all soft tissue
sarcomas. Typically, the tumor arises in young adult extremities in
their third and fourth decades [8], although a wide range of ages
and locations has been reported. In the extensive review by Chung
and Enzinger [2], the age of patients ranged from 7 to 83 years,
with a median age of 27 years. Only 2% of reported cases were
younger than 10 years old.
Clinically, it often presents as a painless slow growing mass
that has been present for several months or even years [9]. Median
duration of symptoms until the time of diagnosis is 18 months [9,
10]. One of the main differential diagnoses of clear cell sarcoma
is metastatic malignant melanoma [11]. Recently, molecular genetic
characterization of clear cell sarcoma has shown a specific
t(12;22) chromosome translocation not present in cutaneous
malignant melanoma [12, 13]. The prognosis is poor with a mortality
rate ranging from 37% to 59% in the largest series [2, 9]. In the
Mayo Clinic series, the survival at 5, 10 and 20 years was 67%, 33%
and 10%, respectively [10]. Tumor size (greater than 5 cm) in
particular, necrosis, and local recurrence are unfavourable
prognostic factors [4]. Early diagnosis and initial radical surgery
are essential for a favourable outcome. Once regional lymph node
metastases or hematogenous dissemination has occurred, the
prognosis is dismal. Complete tumor resection represents the
mainstay of treatment and is the sole treatment for patients with
small tumors. Aggressive surgical resection with wide margins is
warranted to decrease local recurrences. When conservative complete
excision is not feasible, mutilating surgery should be considered.
In patients with complete excision, adjuvant treatment seems
unnecessary [14]. Radiotherapy is strongly suggested for close
resection margins [9, 15]. Chemotherapy is predominantly employed
in case of disseminated disease [15, 16].
There is, as yet, no answer to the question of prophylactic
regional lymph node dissection [17]. Some authors recommend
prophylactic elective regional lymph node dissection as part of the
therapy [4] where others suggest using lymphadenectomy only in case
of clinical lymphadenopathy [14].
The concept of SLNB represents a major new opportunity to
stratify patients for appropriate surgery in cancer [18, 19]. Now
lymphoscintigraphy for sentinel node identification has been
extensively validated in breast cancer and melanoma [20-23]. Whilst
most data have been collected from patients suffering from
carcinoma of the breast or skin (melanoma), there is increasing
interest in other areas [24]; a recent review by Michl et al. [25]
has reported the possible use of the SLNB technique in various skin
malignancies such as squamous cell carcinoma, Merkel cell
carcinoma, adnexal carcinoma and others.
Up to date there are no reports regarding the potential use of
the SLNB technique in clear cell sarcoma. However, given its
aggressiveness and frequent metastasization to locoregional
lymphnodes, it seemed appropriate to use this method in this case
as well. Prophylactic lymphadenectomy is, at present, not advised
in treating clear cell sarcoma [15], while it is used where
lymphnode metastases are clinically or instrumentally detectable.
The early detection of occult lymphatic metastases by lymphatic
mapping and SLNB could also be useful in patients affected with
clear cell sarcoma. In our case described here, the patient did not
have palpable adenopathy and instrumental examination (CT scan and
ecotomography) was negative in locoregional lymph nodes. However, a
lymph node biopsy clearly showed lymphnode metastasis, thus
allowing early lymphadenectomy to the axilla. Even if this result
should be confirmed by wider studies, we want to highlight the
feasibility of this method also in cases of particularly aggressive
and potentially lethal tumours like clear cell sarcoma, as yet
undocumented.
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