Merkel cell carcinoma: extended lymphadenectomy and reconstruction with biosynthetic prosthesis

Merkel cell carcinoma: extended lymphadenectomy and reconstruction with biosynthetic prosthesis Introduction: Merkel cell carcinomas (MCC) is an aggressive neuroendocrine carcinoma originating from the Merkel cell in the dermo-epidermal junction. Only 10% of MCC occur on the skin of the trunk. Case report: We report a case of Merkel’s abdominal carcinomas treated with extensive inguinal lymphadenectomy and ® ® reconstruction of the abdominal wall and inguinal canal using prosthesis GORE BIO-A . Discussion: Immunohistochemical analysis by tumor-specific markers is crucial for diagnosis and permits differentiation from other tumors of the skin. MCC is an aggressive tumor with poor prognosis. Conclusions: For primary tumors without indications of the presence of organ metastases complete surgical excision is the gold standard. Gore BIO-A is a biosynthetic prosthesis with manageable structure that allows it to be positioned and shaped according to needs, its strength provides for excellent support for the reconstruction of the inguinal canal wall. INTRODUCTION (UV). MCC is closely associated with squamous cell carcinoma Freidrich Sigmund Merkel first described the Merkel cell in (SCC), basal cell carcinoma (BCC) and Bowen’s disease (a first, superficial SCC variant), all of which are most frequently 1875. Merkel cell carcinoma (MCC) was originally described by Toker in 1972 as trabecular carcinoma of the skin [1]. caused by exposure to UV rays. An increase in MCC incidence has also been observed in people with chronic immune sup- MCC is an aggressive neuroendocrine carcinoma originating from the Merkel cell in the dermo-epidermal junction, which pression [3]. Feng et al. [4] scientists at the University of Pittsburgh dis- belongs to the amine precursor uptake and decarboxylation (APUD) system. covered a ‘Merkel cell polyomavirus’ MCV o McPyV which may be a contributing factor to MCC pathogenesis. High The median age at diagnosis is ~65 years. Incidence is con- siderably greater in whites than blacks and slightly greater in levels of viral DNA and clonal integration of the virus in MCC tumors have also been reported along with expression of males than females [2]. The exact aetiology is unknown. Scientific evidence also certain viral antigens in MCC cells and the presence of anti- viral antibodies. shows a strong link between MCC and ultraviolet light exposure Received: March 13, 2018. Accepted: April 26, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 E. Coppola Bottazzi et al. Figure 1: PET and CT scan of right breast nodule (A and A1), left abdominal mass (B and B1) and left inguinal lymph node (C and C1). Approximately 50% of MCCs occur on the head and neck (46% of these in the periorbital region), 35% on the extremities and 10% on the skin of the trunk [5]. We report a case of Merkel’s abdominal carcinomas treated with extensive inguinal lymphadenectomy and reconstruction of the abdominal wall and inguinal canal using biosynthetic prosthesis. CASE REPORT A 58-year-old man, with a recent history of polyglobuline trea- ted with phlebotomies, first degree obesity and multinodal goi- ter. After the appearance of a subcutaneous swelling of the left abdominal wall, extended by the umbilical line for ~6 cm, of solid consistency and fixed to the fascia, ultrasound imaging and a biopsy of the removal of the abdomen were performed. Histological examination described the presence of a malig- nant neoplasia of medium-sized cells, with nucleated chroma- tin, dispersed in nests and cords; with high mitotic activity and apoptotic index. Immunohistochemical study was positive for Citokeratina (CK) 20, CKpan, CD56, chromogranin, sinaptofisine, CD44, neurofilaments (dot-like, partial) and Pax5 (weak and partial) and negative for Vimentina, S100, CK7, CD117, CD99, TdT and thyroid transcription factor 1 (TTF1). Diagnosis was of Merkel’s skin neuroendocrine carcinoma. A positron emission tomography (PET) with CT-scan recon- struction showed hyper metabolic at the right breast nodule of 1.8 cm (SUV 3.7), at the left abdominal mass of 6 cm (SUV 9.37) and at superficial (SV 9.6) and deep (SUV 8.5) inguinal lymph node (Fig. 1). Then a right mastectomy with third level axillary lymphade- nectomy, for sentinel lymph node positivity; removal of the left Figure 2: Inguinal region after linfoadenectomy (N, nerve; V, Vein; A, Artery; abdominal mass with resection of the band of external oblique *spermatic cord). muscle and of the infiltrated anterior inguinal wall; lymphade- nectomy of the external and common left iliac artery; deep and superficial left inguinal lymphadenectomy (Fig. 2) were per- Final histological examination confirmed an abdominal formed. Reconstruction of the abdominal band and of the MCC with inguinal and axillary massive lymph node metastasis ® ® inguinal canal wall was obtained using a GORE BIO-A pros- T3 N1b M1a, Stage IV. The right breast nodule was a melanocy- thesis (Fig. 3). tic nerve. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Merkel cell carcinoma: extended lymphadenectomy and reconstruction 3 Figure 4: Gore Bio-A tissue reinforcement and 3D aspect (Images provided cour- tesy of W. L. Gore & Associates.). barrier a stimulation of collagens deposition and ingrowths of ® ® Figure 3: GORE BIO-A prosthesis used for reconstruction of the abdominal new connective soft tissue occurs (Fig. 4)[10]. wall and inguinal canal. Gore BIO-A has a manageable structure that allows it to be positioned and shaped according to needs, its strength provides According to the oncologists and radiotherapists, the patient for excellent support for the reconstruction of the inguinal was not subjected to any therapy; 10-month follow-up there is canal wall (Fig. 3) as in our case, where the front wall of the no evidence of recurrence. We did not observe inguino-crural inguinal canal and the external inguinal ring have been hernias after 10 months. removed for neoplastic infiltration. DISCUSSION CONCLUSION Immunohistochemical analysis by tumor-specific markers is MCC is an aggressive tumor with poor prognosis. For primary crucial for diagnosis and permits differentiation from other tumors without indications of the presence of organ metasta- tumors of the skin. MCC is positive for Cytokeratin (CK) 20, ses complete surgical excision is the gold standard. When neuro-filament and neuron specific enolase (NSE); negative to micrometastases are found in the sentinel lymph node, this CK 7, Thyroid Transcription Factor (TTF) 1, S100 Protein, leuko- should be followed by complete lymphadenectomy. MCCs are cyte commion agent (LCA) whereas melanoma is only positive usually radiosensitive; Retrospective analyses show that the for S100 protein and Lymphoma only for LCA [6]. high local recurrence rate after R0 surgery of the primary tumor Fine needle aspiration cytology (FNAC) enables an early alone can be reduced significantly by combined loco regional noninvasive diagnosis of this aggressive tumor to facilitate adjuvant radiation therapy. In advanced cases with radiological early planning for surgery [7]. evidence of lymph node diffusion, as in our case, surgical resec- Loco regional lymph nodes represent the most frequent tion and extensive loco regional lymphadenectomy are spread of MCC, highly predictive of negative prognosis tumor. indicated. Sentinel lymph nodes (SLN) should only be used in patients without clinical evidence of lymph node involvement, enabling a correct staging of the disease and imposing a more invasive CONFLICT OF INTEREST STATEMENT surgical step only in patients who show lymph node metasta- None declared. ses. The sentinel lymph node appears to be a specific and sen- sitive prognostic index in the MCC [8]. Surgery is the mainstay of treatment for MCC. Wide local REFERENCES excision with 1–2 cm margins to the investing fascia layer remains the standard surgical technique. Radiotherapy is an 1. Toker C. Trabecular carcinoma of the skin. Arch Dermatol inferior option for cancer control, since the complete response 1972;105:107–10. is only 75% [9]. 2. Agelli M, Clegg LX. Epidemiology of primary Merkel cell car- Gore Bio-A tissue reinforcement is a 3D web of completely cinoma in the United States. J Am Acad Dermatol 2003;49: absorbable synthetic polymers replaced by soft tissue within 6 832–41. months; It is a mix of glycolic acid and trimethylene carbonate 3. Houben R, Schrama D, Becker JC. Molecular pathogenesis of and its function consists of rather than producing a mechanical Merkel cell carcinoma. Exp Dermatol 2009;18:193–8. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018 4 E. Coppola Bottazzi et al. 4. Feng H, Shuda M, Chang Y, Moore PS. Clonal integration of 7. Tai P. A practical update of surgical management of merkel a polyomavirus in human Merkel cell carcinoma. Science cell carcinoma of the skin. ISRN Surg 2013;2013:850797. 2008;319:1096–1100. 8. Migliano E, Monarca C, Tedesco M, Rizzo MI, Bucher S. 5. Albores-Saavedra J, Batich K, Chable-Montero F, Sagy N, [Merkel cell carcinoma and sentinel lymph node dissection: Schwartz AM, Henson DE. Merkel cell carcinoma demo- nine cases report]. G Chir 2008;29:28–32. graphics, morphology, and survival based on 3870 cases: a 9. Bichakjian CK, Olencki T, Alam M, Andersen JS, Berg D, population based study. J Cutan Pathol 2010;37:20–7. Bowen GM, et al. Merkel cell carcinoma, version 1.2014. 6. Busse PM, Clark JR, Muse VV, Liu V. Case records of the J Natl Compr Canc Netw 2014;12:410–24. Massachusetts General Hospital. Case 19-2008. A 63-year- 10. Sutton PA, Evans JP, Uzair S, Varghese JV. The use of Gore old HIV-positive man with cutaneous Merkel-cell carcin- Bio-A in the management of the open abdomen. BMJ Case oma. N Engl J Med 2008;358:2717–23. Rep 2013;1–2. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Merkel cell carcinoma: extended lymphadenectomy and reconstruction with biosynthetic prosthesis

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Abstract

Introduction: Merkel cell carcinomas (MCC) is an aggressive neuroendocrine carcinoma originating from the Merkel cell in the dermo-epidermal junction. Only 10% of MCC occur on the skin of the trunk. Case report: We report a case of Merkel’s abdominal carcinomas treated with extensive inguinal lymphadenectomy and ® ® reconstruction of the abdominal wall and inguinal canal using prosthesis GORE BIO-A . Discussion: Immunohistochemical analysis by tumor-specific markers is crucial for diagnosis and permits differentiation from other tumors of the skin. MCC is an aggressive tumor with poor prognosis. Conclusions: For primary tumors without indications of the presence of organ metastases complete surgical excision is the gold standard. Gore BIO-A is a biosynthetic prosthesis with manageable structure that allows it to be positioned and shaped according to needs, its strength provides for excellent support for the reconstruction of the inguinal canal wall. INTRODUCTION (UV). MCC is closely associated with squamous cell carcinoma Freidrich Sigmund Merkel first described the Merkel cell in (SCC), basal cell carcinoma (BCC) and Bowen’s disease (a first, superficial SCC variant), all of which are most frequently 1875. Merkel cell carcinoma (MCC) was originally described by Toker in 1972 as trabecular carcinoma of the skin [1]. caused by exposure to UV rays. An increase in MCC incidence has also been observed in people with chronic immune sup- MCC is an aggressive neuroendocrine carcinoma originating from the Merkel cell in the dermo-epidermal junction, which pression [3]. Feng et al. [4] scientists at the University of Pittsburgh dis- belongs to the amine precursor uptake and decarboxylation (APUD) system. covered a ‘Merkel cell polyomavirus’ MCV o McPyV which may be a contributing factor to MCC pathogenesis. High The median age at diagnosis is ~65 years. Incidence is con- siderably greater in whites than blacks and slightly greater in levels of viral DNA and clonal integration of the virus in MCC tumors have also been reported along with expression of males than females [2]. The exact aetiology is unknown. Scientific evidence also certain viral antigens in MCC cells and the presence of anti- viral antibodies. shows a strong link between MCC and ultraviolet light exposure Received: March 13, 2018. Accepted: April 26, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 E. Coppola Bottazzi et al. Figure 1: PET and CT scan of right breast nodule (A and A1), left abdominal mass (B and B1) and left inguinal lymph node (C and C1). Approximately 50% of MCCs occur on the head and neck (46% of these in the periorbital region), 35% on the extremities and 10% on the skin of the trunk [5]. We report a case of Merkel’s abdominal carcinomas treated with extensive inguinal lymphadenectomy and reconstruction of the abdominal wall and inguinal canal using biosynthetic prosthesis. CASE REPORT A 58-year-old man, with a recent history of polyglobuline trea- ted with phlebotomies, first degree obesity and multinodal goi- ter. After the appearance of a subcutaneous swelling of the left abdominal wall, extended by the umbilical line for ~6 cm, of solid consistency and fixed to the fascia, ultrasound imaging and a biopsy of the removal of the abdomen were performed. Histological examination described the presence of a malig- nant neoplasia of medium-sized cells, with nucleated chroma- tin, dispersed in nests and cords; with high mitotic activity and apoptotic index. Immunohistochemical study was positive for Citokeratina (CK) 20, CKpan, CD56, chromogranin, sinaptofisine, CD44, neurofilaments (dot-like, partial) and Pax5 (weak and partial) and negative for Vimentina, S100, CK7, CD117, CD99, TdT and thyroid transcription factor 1 (TTF1). Diagnosis was of Merkel’s skin neuroendocrine carcinoma. A positron emission tomography (PET) with CT-scan recon- struction showed hyper metabolic at the right breast nodule of 1.8 cm (SUV 3.7), at the left abdominal mass of 6 cm (SUV 9.37) and at superficial (SV 9.6) and deep (SUV 8.5) inguinal lymph node (Fig. 1). Then a right mastectomy with third level axillary lymphade- nectomy, for sentinel lymph node positivity; removal of the left Figure 2: Inguinal region after linfoadenectomy (N, nerve; V, Vein; A, Artery; abdominal mass with resection of the band of external oblique *spermatic cord). muscle and of the infiltrated anterior inguinal wall; lymphade- nectomy of the external and common left iliac artery; deep and superficial left inguinal lymphadenectomy (Fig. 2) were per- Final histological examination confirmed an abdominal formed. Reconstruction of the abdominal band and of the MCC with inguinal and axillary massive lymph node metastasis ® ® inguinal canal wall was obtained using a GORE BIO-A pros- T3 N1b M1a, Stage IV. The right breast nodule was a melanocy- thesis (Fig. 3). tic nerve. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Merkel cell carcinoma: extended lymphadenectomy and reconstruction 3 Figure 4: Gore Bio-A tissue reinforcement and 3D aspect (Images provided cour- tesy of W. L. Gore & Associates.). barrier a stimulation of collagens deposition and ingrowths of ® ® Figure 3: GORE BIO-A prosthesis used for reconstruction of the abdominal new connective soft tissue occurs (Fig. 4)[10]. wall and inguinal canal. Gore BIO-A has a manageable structure that allows it to be positioned and shaped according to needs, its strength provides According to the oncologists and radiotherapists, the patient for excellent support for the reconstruction of the inguinal was not subjected to any therapy; 10-month follow-up there is canal wall (Fig. 3) as in our case, where the front wall of the no evidence of recurrence. We did not observe inguino-crural inguinal canal and the external inguinal ring have been hernias after 10 months. removed for neoplastic infiltration. DISCUSSION CONCLUSION Immunohistochemical analysis by tumor-specific markers is MCC is an aggressive tumor with poor prognosis. For primary crucial for diagnosis and permits differentiation from other tumors without indications of the presence of organ metasta- tumors of the skin. MCC is positive for Cytokeratin (CK) 20, ses complete surgical excision is the gold standard. When neuro-filament and neuron specific enolase (NSE); negative to micrometastases are found in the sentinel lymph node, this CK 7, Thyroid Transcription Factor (TTF) 1, S100 Protein, leuko- should be followed by complete lymphadenectomy. MCCs are cyte commion agent (LCA) whereas melanoma is only positive usually radiosensitive; Retrospective analyses show that the for S100 protein and Lymphoma only for LCA [6]. high local recurrence rate after R0 surgery of the primary tumor Fine needle aspiration cytology (FNAC) enables an early alone can be reduced significantly by combined loco regional noninvasive diagnosis of this aggressive tumor to facilitate adjuvant radiation therapy. In advanced cases with radiological early planning for surgery [7]. evidence of lymph node diffusion, as in our case, surgical resec- Loco regional lymph nodes represent the most frequent tion and extensive loco regional lymphadenectomy are spread of MCC, highly predictive of negative prognosis tumor. indicated. Sentinel lymph nodes (SLN) should only be used in patients without clinical evidence of lymph node involvement, enabling a correct staging of the disease and imposing a more invasive CONFLICT OF INTEREST STATEMENT surgical step only in patients who show lymph node metasta- None declared. ses. The sentinel lymph node appears to be a specific and sen- sitive prognostic index in the MCC [8]. Surgery is the mainstay of treatment for MCC. Wide local REFERENCES excision with 1–2 cm margins to the investing fascia layer remains the standard surgical technique. Radiotherapy is an 1. Toker C. Trabecular carcinoma of the skin. Arch Dermatol inferior option for cancer control, since the complete response 1972;105:107–10. is only 75% [9]. 2. Agelli M, Clegg LX. Epidemiology of primary Merkel cell car- Gore Bio-A tissue reinforcement is a 3D web of completely cinoma in the United States. J Am Acad Dermatol 2003;49: absorbable synthetic polymers replaced by soft tissue within 6 832–41. months; It is a mix of glycolic acid and trimethylene carbonate 3. Houben R, Schrama D, Becker JC. Molecular pathogenesis of and its function consists of rather than producing a mechanical Merkel cell carcinoma. Exp Dermatol 2009;18:193–8. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018 4 E. Coppola Bottazzi et al. 4. Feng H, Shuda M, Chang Y, Moore PS. Clonal integration of 7. Tai P. A practical update of surgical management of merkel a polyomavirus in human Merkel cell carcinoma. Science cell carcinoma of the skin. ISRN Surg 2013;2013:850797. 2008;319:1096–1100. 8. Migliano E, Monarca C, Tedesco M, Rizzo MI, Bucher S. 5. Albores-Saavedra J, Batich K, Chable-Montero F, Sagy N, [Merkel cell carcinoma and sentinel lymph node dissection: Schwartz AM, Henson DE. Merkel cell carcinoma demo- nine cases report]. G Chir 2008;29:28–32. graphics, morphology, and survival based on 3870 cases: a 9. Bichakjian CK, Olencki T, Alam M, Andersen JS, Berg D, population based study. J Cutan Pathol 2010;37:20–7. Bowen GM, et al. Merkel cell carcinoma, version 1.2014. 6. Busse PM, Clark JR, Muse VV, Liu V. Case records of the J Natl Compr Canc Netw 2014;12:410–24. Massachusetts General Hospital. Case 19-2008. A 63-year- 10. Sutton PA, Evans JP, Uzair S, Varghese JV. The use of Gore old HIV-positive man with cutaneous Merkel-cell carcin- Bio-A in the management of the open abdomen. BMJ Case oma. N Engl J Med 2008;358:2717–23. Rep 2013;1–2. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy098/4996200 by Ed 'DeepDyve' Gillespie user on 21 June 2018

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Journal of Surgical Case ReportsOxford University Press

Published: May 15, 2018

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