Atypical Presentation of Merkel Cells Carcinoma
in Thigh. An Unusual Case
Andrés Limardo1*, Gustavo Prats1, Adrían Ortega1 and Rubén Padín1,2
1Surgeon of Head and Neck, National Hospital Prof. A. Posadas, Argentina
2Chief of Head and Neck Surgery, National Hospital Prof. A. Posadas, Argentina
Submission: January 30, 2017; Published: May 30, 2017
*Corresponding author: Andrés Constantino Limardo, Surgeon of Head and Neck, National Hospital Prof. A. Posadas, Argentina,
Tel: 54911-53149515; Email: firstname.lastname@example.org
How to cite this article: Limardo A, Prats G, Ortega A, Padín R. Atypical Presentation of Merkel Cells Carcinoma in Thigh. An Unusual Case. JOJ Case Stud. 2017; 2(5) : 555598. DOI:10.19080/JOJCS.2017.02.555598.
Background: The Merkel Cells Carcinoma (MCC) is a neuroendocrine carcinoma of the skin. MCC within the lymph nodes in the absence of a primary site is rare and has only been reported sporadically
Case report: A male patient of 62 years old consults by right inguinal tumor. Lymphadenectomy is made and it informs carcinoma of cells of Merkel. Hidden primary tumor. After 20 months of follow up appears injury to nodular in internal face of right thigh interpreted like primary tumor. Resection is made. We discuss the disease, the diagnosis and the treatment of these tumors.
Conclusion: The Merkel Cells Carcinoma (MCC) is a neuroendocrine carcinoma of the skin. MCC within the lymph nodes in the absence of a primary site is rare and has only been reported sporadically. Our case may represent a lymph node metastasis from an occult or regressed skin primary, but we cannot preclude the possibility of a primary nodal tumor. It is not known until today as it is “the best” therapeutic choice for these cases
Keywords: Tumor of cells of Merkel; Carcinoma of cells of Merkel; Rare Tumors of soft parts .
Merkel Cell Carcinoma (MCC) is a neuroendocrine carcinoma of the skin. Although it is 40 times less common that melanoma malignant has mortality greater than this one, with a 30% in the CCM as opposed to a 15% in melanoma . MCC within the lymph nodes in the absence of a primary site is rare and has only been reported sporadically . A case of tumor of cells of Merkel appears next that it make debut in form hides and after 20 months the diagnosis could be confirmed. We discuss the disease, the diagnosis and the treatment of these tumors.
A male patient of 62 years old without relevance antecedents, consult by stony hard tumor in right inguinal region of 2 months of evolution. To the physical examination reveal a mobile and superficial tumor of approximately 35x25mm. It seems inguinal node. The rest of the physical examination in the genital, anal, gluteal zone and inferior tight does not present alterations. Magnetic Resonance Imaging (MRI) of abdomen, pelvis and thighs is realized. It reveals iso intense tumor to muscle in T1-weighted and mildly hyper intense tumor in T2-weightedof 39x28mm in right inguinal region on subcutaneous cellular weave. STIR sequences have high signal intensity. Images around are interpreted like normal nodes (Figure 1). Normal Fibro colonoscopy.
It impresses a neuroectodermic origin with neoplasia of Merkel for which it is necessary Immunohistochemical study to confirm the diagnosis. Surgery is decided. Biopsy by frozen section is made with general anesthesia that informs atipic cells. Inguinal lymphadenectomy is completed.
Cromogranin, sinaptofibrin, CD56 and 20CK 20positive. The
injury is interpreted as metastatic carcinoma of cells of Merkel
with hidden primary tumor. Reinforcement with 5800cCy is
made by Department of Oncology. After 20 months of follow up it
appears a violet nodule on skin in internal face of the right thigh
that is interpreted like primary tumor of Merkel.
FNA is decided that suspicious tumor informs Merkel
cells carcinoma with the antecedents. Surgery is decided.
Resection with margins is made, 5cm lateral and 3cm deep and
reconstruction with skin local flap (Figure 3). Histology reveals
Merkel cells carcinoma confirmed by Immunohistochemical
The MCC appears in people with an average age of 70 years,
although several cases of MCC have been described in which
the age was inferior to the awaited one. It is more frequent in
people of white skin. According to some authors it predominates
in male patients . Sun exposure is thought to play a major
role in pathogenesis. It has been observed in many cases that
these patients were put under immunosuppression after the
transplant of a solid organ, or treated by aplastic anemia,
with antithymocyte globulin and cyclosporine . Merkel cell
polyomavirus (MCV) is detected in 50% to 100% of patient
samples. The tumor is located in the dermis, and can extend
to the subcutaneous cellular weave and deep soft weaves.
Epidermis usually is respected, although it can be ulceror to
show hyperplasia reactivates .
MCC express the markers of epithelial origin and
neuroendocrine type. The MCC express markers of epithelial
origin (CK 20) and neuroendocrine type (NSE, Cromogranin B
and A; neurofilament L and polypeptide M) and TTF1 negative
to differentiate these tumors from metastatic small-cell lung
cancer .It has been used for the diagnosis of the tumor the
cytology by fine needle aspiration .
Clinically the MCC appears typically like primary a cutaneous
injury (75%), although it can make debut as a metastatic
disease . There are currently two possible explanations for
what occurred in our patient. First, the inguinal mass could
be a massively enlarged lymph node where precursor cells
became neoplastic. This would be consistent with a presumptive
diagnosis of primary nodal disease. Alternatively, an initial skin
lesion could have spontaneously regressed and the inguinal
mass represents a single site of metastasis. After 20 months of
follow up it appears a lesion on skin in internal face of the right
thigh that is interpreted like primary tumor of Merkel. So we
concluded the patient had MCC with inguinal nodes metastasis.
But it was an atypical presentation. The primary injury usually
is painless, sobrehigh, of fast growth. That appears like a solitary
red-violet nodule or papule with telangiectasia in surface, reason
. Tumors sizes oscillates between 0.5 and 5cm (generally,
minor of 2cm) and usually are not ulcer. It can evolve badly to a
In the presence of a nodal Merkel cell tumor, an exhaustive
clinico-radiologic search for a primary tumor must be carried
out . MRI of abdomen, pelvis and thighs is realized and Fibro
colonoscopy was made in our patient.
To respect to the surgery three subjects are due to discuss:
the extension of margins, sentinel lymph node biopsy, as well as nodes dissection. Recommends margins of 3cm and depth
2cm when it is possible . The Mohs surgery followed of
radiotherapy are used in small injuries . Are data no available
on the utility of the study of the sentinel lymph node in these
The sentinel lymph node biopsy is recommended in equal
or greater injuries of 1 cm, by the risk to nodal metastatic and
with node dissection in case of positive. With respect to the
Radiotherapy, we know that they are sensible tumors. It have
been documented complete answer of the measurable tumor of
96%, and 4% of partial answer (rate global answer of the 100%)
. The adjuvant Radiotherapy has shown to improve local
recurrence and average survival. It does not seem necessary
in the cases where the resection is complete, although it is
recommended in the cases with affected margins, incomplete
extirpation or in cases in which the surgery accomplishment
is impossible. The use of adjuvant radiotherapy or as only
treatment in non-surgical stages has demonstrated to increase
the survival . The chemotherapy in this tumor does not
increase the survival and causes considerable toxicity, advising
itself solely in patients with remote metastatic where the loco
regional treatment has not controlled. Immunotherapy targeting
the programmed cell death receptor 1/programmed cell death
ligand1 (PD-1/PD-L1) checkpoint holds great promise in
treating advanced MCC and may provide durable responses in a
portion of patients .Despite, the handling individualized in
each patient is recommended .
The Merkel Cells Carcinoma (MCC) is a neuroendocrine
carcinoma of the skin. MCC within the lymph nodes in the absence
of a primary site is rare and has only been reported sporadically.
Our case may represent a lymph node metastasis from an occult
or regressed skin primary, but we cannot preclude the possibility
of a primary nodal tumor. It is not known until today as it is “the
best” therapeutic choice for these cases.
Andrés C. Limardo declares that he has no conflict of interest,
Gustavo Prats declares that he has no conflict of interest, Adrían
Ortega declares that he has no conflict of interest, Rubén Padín
declares that he has no conflict of interest.
Ethical approval (humans): All procedures performed
in studies involving human participants were in accordance
with the ethical standards of the institutional and/or national
research committee and with the 1964 Helsinki declaration and
its later amendments or comparable ethical standards.
Ethical approval (animals): This article does not contain
any studies with animals performed by any of the authors.
Informed consent: Informed consent was obtained from
all individual participants included in the study.