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Open Medicine

formerly Central European Journal of Medicine

Editor-in-Chief: Darzynkiewicz, Zbigniew


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Volume 14, Issue 1

Issues

Volume 10 (2015)

Familial polyposis coli: the management of desmoid tumor bleeding

Armando Calogero
  • Corresponding author
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Caterina Sagnelli
  • Department of Mental Health and Public Medicine, University of Campania Luigi Vanvitelli, Naples, Italy
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/ Nicola Carlomagno
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Vincenzo Tammaro
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Maria Candida
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Antonio Vernillo
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Gaia Peluso
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Gianluca Minieri
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Michele Santangelo
  • General Surgery and Transplant Unit, Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini, 80131 Naples, Italy
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/ Concetta Anna Dodaro
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Published Online: 2019-07-19 | DOI: https://doi.org/10.1515/med-2019-0064

Abstract

Background

There is currently no standard treatment for desmoid tumors (DTs) associated with familial polyposis coli (FAP). Familial adenomatous polyposis in DT patients is sometimes a life-threatening condition.

Methods

We enrolled all consecutive patients with FAP treated at Unit of General Surgery and Transplant, University of Naples Federico II and evaluated the incidence of DTs on FAP between 1996 and 2016.

Results

We observed 45 consecutive patients with FAP; of these 5 were DT-FAP-associated. All 5 cases with FAP were young women, age 25 to 65 years, previously treated by colectomy. Of these, 4 patients presented a parietal localization and had been treated with a wide surgical exeresis; one patient had an intra-abdominal, mesenteric tumor that was unresectable at laparotomy. We performed CT-guided drainage, ureteral stenting, medical therapy (sulindac+tamoxifene), and chemotherapy (dacarba-zine+doxorubicine).

All patients were alive and underwent follow-ups for 5 years post-surgery; only 1 patient with parietal localization showed a local relapse after 2 years.

Conclusions

We propose a modulated approach to the single patient with FAP, with surgery as treatment of choice for parietal localization disease and integrating different kinds of therapies (surgery alone or associated with RT, CT) for the intra-abdominal tumor.

Keywords: Desmoid tumor; Familial adenomatous polyposis; Radiotherapy; Surgery

1 Background

In 1838 Johannes Mueller first described a desmoid tumor (DT) as a neoplasia arising from tendons, bandlike in shape, with local aggressiveness and possibility of recurrence [1]. Patients with familial adenomatous polyposis (FAP) may develop soft-tissue tumors, DTs. These tumors are considered benign but can be life threatening through progressive enlargement and consequently, pressure on gastrointestinal or urinary tracts, nervous or vascular systems.

DTs can arise in the mesentery, abdominal wall, or areas of scars; they are considered a benign entity but can cause severe morbidity and mortality because of their progressive growth that threatens the intra-abdominal organs, nerves, and vessels [2]. They occur in about 10% to 25% of FAP patients, commonly in women, and are one of the most important causes of death after colectomy [3].

FAP is an inherited syndrome that, with other similar neoplasms such as Lynch syndrome, MUTYH-associated polyposis (MAP), and several hamartomatous polyposis conditions, accounts for about 3% to 6% of all colorectal cancers [3, 4, 5, 6, 7]. The etiopathogenesis is still unknown, but there are different hypotheses, including genetic alterations [8,9] and/or an endocrinological or traumatic genesis [10, 11, 12].

However, several doubts about therapy and management of FAP have been reported [13].The surgical approach is the first therapeutic step, but the unfavorable biologic characteristics of desmoids do not always allow a wide excision; therefore, radiotherapy and chemotherapy have been tested as the primary definitive treatment or as an adjuvant therapy after surgery. Recently, several authors reported a cell-based therapeutic approach. A therapy with progenitors like endothelial progenitor cells (EPCs), using intracellular signals, may impair the adverse tumor vascularization [14, 15, 16, 17, 18, 19, 20]. In particular, the role of vascular endothelial growth factor (VEGF) and its calcium-mediated signaling as a possible target site of anti-angiogenetic therapy in EPCs has been addressed [21, 22, 23, 24, 25, 26, 27, 28, 29, 30].

2 Methods

We enrolled all the consecutive patients with FAP who underwent surgery at the Unit of General Surgery and Transplant, University of Naples Federico II and evaluated the incidence of DTs on FAP between years 1996 and 2016. All procedures applied were in accordance with international guidelines, with the standards of the local ethics committees, and with the Helsinki Declaration of 1975, 1983 revision. At the first appointment, each patient signed informed consent for the surgical procedure and collection and use in clinical research of the data obtained, as established by the Ethics Committee of AOU-University of Naples Federico II.

All patients at admission submitted to blood exams, ultrasound, CT Scan, and fine-needle cytology (FNC) in accordance with good clinical practice. The safety and the efficacy of surgical treatment were assessed. All patients were re-evaluated by periodic follow-ups, including examination by a physician, chest X-ray, and computed tomography abdominopelvic or magnetic resonance imaging every 3 months for the first year, every 6 months for the following 4 years, and subsequently every year.

3 Results

During the ten years 1996 to 2016 we observed 45 consecutive patients with FAP; of these 5 presented with both FAP and DT. These 5 patients were all female, aged between 25 and 65 years old, and underwent surgery in the previous 3 to 6 years for FAP: 1 underwent total colectomy combined with ileo-rectal anastomosis (IRA) and 4 received a proctocolectomy with ileal pouch-anal anastomosis (IPAA). The demographic and clinical characteristics of the 5 patients with FAP and DT are shown in Table 1. The desmoids were located in 2 patients who were members of the same family, at rectus abdominis; in 2 cases at the external oblique muscle ,and at the mesentery in 1 patient. (Table 1)

Table 1

Demographic and clinical presentation of the 5 patients with FAP and desmoid tumor

The patients with parietal tumor showed no symptoms; they referred only to the onset of a movable and painless abdominal mass. One of the desmoids found in the external oblique muscle showed bleeding, but without anemia. The patient with the mesenteric desmoid reported abdominal pain, weight loss, and sub-occlusive crises, associated with left hydronephrosis.

Surgical treatment was possible in all parietal locations, in the form of wide exeresis. (Table 1, Figure 1). The patients were discharged 6 to 8 days after surgery without complications. The mesenteric desmoid was unresectable at laparotomy because of its connections with the great abdominal vessels and small bowel loops. Therefore, the patient underwent TC-guided drainage and ureteral stenting to treat the left hydronephrosis and was treated with tamoxifen and sulindac.

A: Desmoid tumor that arose after total colectomy in the anterior abdominal wall in a young FAP-affected female patient: CT scan. B: Surgical specimen of desmoid tumor that arose after total colectomy in the anterior abdominal wall in a young FAP-affected female patient.
Figure 1

A: Desmoid tumor that arose after total colectomy in the anterior abdominal wall in a young FAP-affected female patient: CT scan.

B: Surgical specimen of desmoid tumor that arose after total colectomy in the anterior abdominal wall in a young FAP-affected female patient.

One patient received post-operative radiotherapy, whereas the others followed an adjuvant therapy with sulindac and tamoxifen in the year after the operation. After 1 year of follow-up, the patient was alive and in good health, but the tumor had not regressed. Therefore, chemotherapy with doxorubicin plus dacarbazine was performed; she showed a partial response. At 3 years after the initial diagnosis, she is alive with a tumoral regression shown on CT scan and DMSA scintigraphy. (Table 2)

Table 2

Therapeutic choices

The incidence of desmoid tumors developed in patients with FAP in our series is 11.1%.

All patients were alive and underwent follow-up for 5 years; only 1 patient with an external oblique muscle localization showed a local relapse after 2 years.

4 Conclusion

The DT represents an unfortunate event for patient with FAP and is the third most frequent cause of death in this patient setting. The management of DT is not yet standardized, and it can be different in case of parietal or intra-abdominal locations. Our experience shows how the treatment of these tumors might be particularly challenging. In patients with FAP, the risk of desmoid fibromatosis is increased up to 800-fold compared with the general population; cumulative risk of onset fibromatosis is 16% in the 10 years after colectomy (peak incidence in 1 to 3 years following colorectal surgery) [31]. A surgical approach can be justified for the parietal locations followed by drugs or radiotherapy with a low recurrence rate [32].

In patients with intrabdominal tumors, a modulated therapeutic approach (surgery alone or associated with RT, CT), or also an abstensionist behavior can be justified for the unpredictable natural history of this disease, characterized sometimes by spontaneous regressions and absence of growth of tumor for a long period; even though it has been reported that these lesions have an higher rate of recurrence when associated with FAP [33].

The role of radiotherapy (RT) is yet not clear. Several studies reported the RT efficacy (partial or complete tumoral regression), using 50–60 Gy, with a local control in 89% and recurrences in 20% to 30% [34, 35]. Successes could depend on menopausal induction and the consequential estrogenic decrease in women. This hypothesis is also supported by the low radiosensitivity observed in men.

In our study, we observed only 5 patients with FAP and DT among the 45 FAP patients enrolled. In all parietal tumors, a wide exeresis was possible with safe margins and a minimal rate of recurrence. The surgical treatment of desmoids offers some considerations about the possibility of aggressive procedures and the extension of exeresis, when feasible (Figure 1). The biological characteristics of desmoids do not always allow a perfect exeresis because the nearest structures can be strongly infiltrated at the time of surgery. Thus, it can be necessary to perform a palliative procedure (gastrointestinal bypasses, transureteroureterostomy) [36, 37, 38, 39].

In conclusion, our experience supports a modulated approach to the single patient, with surgery as treatment of choice for parietal localization and integrating different kinds of therapies for the intra-abdominal ones. We indicate early identification of high-risk subjects, such as young women, who are candidates for a restrictive follow-up and an early colectomy. We urge cooperation with National FAP Registers [40].

Abbreviations

FAP:

familial adenomatous polyposis

DT:

desmoid tumor

MAP:

MUTYH-associated polyposis

FNC:

Fine-Needle Cytology

References

  • [1]

    Mueller J, Ueber den feinern bau und forman der Krankhaften geschwulsta, Berlin: G Reimer and Erste Lieferung, 1838: 60 Google Scholar

  • [2]

    Half E, Bercovich D, Rozen P. Familial adenomatous polyposis. Orphanet J Rare Dis. 2009 Oct 12;4:22. doi: 10.1186/1750-1172-4-22 PubMedCrossrefWeb of ScienceGoogle Scholar

  • [3]

    Sturt NJ, Clark SK. Current ideas in desmoid tumours. Fam Cancer. 2006;5(3):275-85; discussion 287-288 CrossrefPubMedGoogle Scholar

  • [4]

    Thirlwell C, Howarth KM, Segditsas S, Guerra G, Thomas HJ, Phillips RK, et al. Investigation of pathogenic mechanisms in multiple colorectal adenoma patients without germline APC or MYH/MUTYH mutations. Br J Cancer 2007,96(11),1729-34, DOI: 10.1038/sj.bjc.6603789 PubMedCrossrefWeb of ScienceGoogle Scholar

  • [5]

    Peluso G, Incollingo P, Calogero A, Tammaro V, Rupealta N, Chiacchio G, et al. Current tissue molecular markers in colorectal cancer: A literature review. Biomed Res Int. 2017;2017:2605628. doi: 10.1155/2017/2605628 Web of SciencePubMedGoogle Scholar

  • [6]

    Truta B, Allen BA, Conrad PG, Weinberg V, Miller GA, Pomponio R, et al. A comparison of the phenotype and genotype in adenomatous polyposis patients with and without a family history, Fam Cancer. 2005, 4, (2), 127-33, DOI: 10.1007/s10689-004-5814-0 PubMedGoogle Scholar

  • [7]

    Bisgaard ML, Fenger K, Bulow S, Niebuhr E, Mohr J. Familial adenomatous polyposis (FAP): frequency, penetrance, and mutation rate. Hum. Mutat. 1994; 3: 121-125 PubMedCrossrefGoogle Scholar

  • [8]

    Carlomagno N, Santangelo ML Mastromarino R, Calogero A, Dodaro C, Renda A. Rare multiple primary malignancies among surgical patients-a single surgical unit experience. Ecancermedicalscience. 2014; 8: 438. PubMedGoogle Scholar

  • [9]

    Roncucci L, Pedroni M, Mariani F. Attenuated adenomatous polyposis of the large bowel: Present and future. World J Gastroenterol. 2017;23(23):4135-4139.  CrossrefPubMedWeb of ScienceGoogle Scholar

  • [10]

    Santangelo ML, Grifasi C, Criscitiello C, Giuliano M, Calogero A, Dodaro C, et al. Bowel obstruction and peritoneal carcinomatosis in the elderly. A systematic review. Aging Clin Exp Res, 2017, 29, (Suppl 1), 73-78, doi: 10.1007/s40520-016-0656-9 PubMedWeb of ScienceCrossrefGoogle Scholar

  • [11]

    Grifasi C, Calogero A, Esposito A, Dodaro C. Perioperative care of elderly outpatient: a review. Ann Ital Chir. 2015;86(2):100-105 PubMedGoogle Scholar

  • [12]

    Desurmont T, Lefevre JH, Shields C, Colas C, Tiret E, Park Y. Desmoid tumour in familial adenomatous polyposis patients: responses to treatments. Fam Cancer, 2015, 14, (1), 31-39 CrossrefPubMedWeb of ScienceGoogle Scholar

  • [13]

    Chittleborough TJ, Warrier SK, Heriot AG, Kalady M, Church J. Dispelling misconceptions in the management of familial adenomatous polyposis. ANZ J Surg. 2017 Jun;87(6):441-445. doi: 10.1111/ans.13919 CrossrefPubMedWeb of ScienceGoogle Scholar

  • [14]

    Shapovalov G, Skryma R, Prevarskaya N. Calcium channels and prostate cancer. Recent Pat. Anticancer Drug. Discov, 2013,8,(1),18e26.11 PubMedGoogle Scholar

  • [15]

    Moccia F, Zuccolo E, Poletto V, Turin I, Guerra G, Pedrazzoli P, et al. Targeting Stim and Orai Proteins as an Alternative Approach in Anticancer Therapy. Curr Med Chem. 2016, 23, (30), 3450-3480,  CrossrefPubMedWeb of ScienceGoogle Scholar

  • [16]

    Dragoni S, Laforenza U, Bonetti E, Reforgiato M, Poletto V, Lodola F, et al. Enhanced expression of Stim, Orai, and TRPC transcripts and proteins in endothelial progenitor cells isolated from patients with primary myelofibrosis. PLoS One 2014,9,(3),e91099, doi: 10.1371/journal.pone.0042541 PubMedWeb of ScienceCrossrefGoogle Scholar

  • [17]

    Dragoni S, Turin I, Laforenza U, Potenza DM, Bottino C, Glasnov TN, et al. Store-operated ca(2+) entry does not control proliferation in primary cultures of human metastatic renal cellular carcinoma. Biomed Res Int, 2014, 2014,739494,  CrossrefWeb of SciencePubMedGoogle Scholar

  • [18]

    Lodola F, Laforenza U, Bonetti E, Lim D, Dragoni S, Bottino C, et al. Store operated Ca2+ entry is remodelled and controls in vivo angiogenesis in endothelial progenitor cells isolated from tumoral patients. PLoS One, 2012,7,(9),e42541 CrossrefGoogle Scholar

  • [19]

    Moccia F. Dragoni S, Lodola F, Bonetti E, Bottino C, Guerra G et al. Store-dependent Ca2+ entry in endothelial progenitor cells as a perspective tool to enhance cell based therapy and adverse tumour vascularization. Curr Med Chem, 2012, 19, 5802-5818, DOI: 10.2174/092986712804143240 CrossrefWeb of ScienceGoogle Scholar

  • [20]

    Moccia F, Lodola F, Dragoni S, Bonetti E, Bottino C, Guerra G, et al. Ca2+ Signalling in endothelial pro genitor cells: a novel means to improve cell-based therapy and impair tumour vascularization, Curr Vasc Pharmacol. 2014, 12, 87-105, DOI: 10.2174/157016111201140327162858 CrossrefGoogle Scholar

  • [21]

    Dragoni S, Reforgiato M, Zuccolo E, Poletto V, Lodola F, Ruffinatti FA et al. Dysregulation of VEGF-induced pro-angiogenic Ca2+ oscillations in primary myelofi-brosis-derived endothelial colony forming cells Exp Hematol. 2015 Dec;43(12):1019-1030.e3. PMID: 26432919 CrossrefGoogle Scholar

  • [22]

    Zuccolo E, Bottino C, Diofano F, Poletto V, Codazzi AC, Mannarino S et al. Constitutive store-operated Ca2+ entry leads to enhanced nitric oxide production and proliferation in infantile hemangioma-derived endothelial colony forming cells. Stem Cells Dev, 2016, 25, (4), 301-19, doi: 10.1089/ scd.2015.0240 Web of ScienceCrossrefGoogle Scholar

  • [23]

    Poletto V, Dragoni S, Lim D, et al. Endoplasmic Reticulum Ca2+ Handling and Apoptotic Resistance in Tumor-Derived Endothelial Colony Forming Cells. J Cell Biochem, 2016, 117, (10), 2260-71, doi: 10.1089/scd.2015.0240 CrossrefWeb of ScienceGoogle Scholar

  • [24]

    Moccia F, Zuccolo E, Poletto V, Cinelli M, Bonetti E, Guerra G, et al. Endothelial progenitor cells support tumour growth and metastatisation: implications for the resistance to anti-an-giogenic therapy. Tumour Biol, 2015, 36, (9), 6603-14. doi: 10.1007/s13277-015-3823-2 CrossrefPubMedGoogle Scholar

  • [25]

    Moccia F, Guerra G. Ca2+ Signalling in Endothelial Progenitor Cells: Friend or Foe? J Cell Physiol, 2016,231,(2), 314-27. doi: 10.1002/jcp.25126 CrossrefWeb of ScienceGoogle Scholar

  • [26]

    Potenza DM, Guerra G, Avanzato D, Poletto V, Pareek S, Guido D et al. Hydrogen sulphide triggers VEGF-induced intracellular Ca2+ signals in human endothelial cells but not in their immature progenitors. Cell Calcium, 2014,56,(3),225-34.  CrossrefWeb of ScienceGoogle Scholar

  • [27]

    Ronco V, Potenza DM, Denti F, Vullo S, Gagliano G, Tognolina M et al. A novel Ca2⁺-mediated cross-talk between endoplasmic reticulum and acidic organelles: implications for NAADP-dependent Ca2⁺ signaling, Cell Calcium. 2015,57,(2),89-100, doi: 10.1016/j.ceca.2015.01.001 CrossrefGoogle Scholar

  • [28]

    Zuccolo E, Dragoni S, Poletto V, Catarsi P, Guido D, Rappa A, et al. Arachidonic acid-evoked Ca2+ signals promote nitric oxide release and proliferation in human endothelial colony forming cells. Vascul Pharmacol, 2016, 87, 159-171.doi: 10.1016/j.vph.2016.09.005 CrossrefPubMedGoogle Scholar

  • [29]

    Rippa E, Altieri F, Di Stadio CS, Miselli G, Lamberti A, Federico A et al. Ectopic expression of gastrokine 1 in gastric cancer cells up-regulates tight and adherens junction proteins network. Pathol Res Pract. 2015, 211, (8), 577-83, doi: 10.1016/j.prp.2015.04.008 Web of ScienceCrossrefPubMedGoogle Scholar

  • [30]

    Di Nezza F, Zuccolo E, Poletto V, Rosti V, De Luca A, Moccia F, et al. Liposomes as a Putative Tool to Investigate NAADP Signaling in Vasculogenesis. J Cell Biochem. 2017. doi: 10.1002/jcb.26019 Web of SciencePubMedGoogle Scholar

  • [31]

    Righetti AEM, Jacomini C, Parra RS, Almeida ALNRd, Rocha JJR, Feres O. Familial adenomatous polyposis and desmoid tumors. Clinics. 2011;66(10): 1839-1842 Web of ScienceCrossrefPubMedGoogle Scholar

  • [32]

    Chittleborough TJ, Warrier SK, Heriot AG, Kalady M, Church J. Dispelling misconceptions in the management of familial adenomatous polyposis. ANZ J Surg. 2017 Jun;87(6):441-445. doi: 10.1111/ans.13919 CrossrefPubMedWeb of ScienceGoogle Scholar

  • [33]

    Dinarvand P, Davaro EP, Doan JV, Ising ME, Evans NR, Phillips NJ, et al. Familial Adenomatous Polyposis Syndrome: An Update and Review of Extraintestinal Manifestations. Arch Pathol Lab Med. 2019 May 9. doi:10.5858/arpa.2018-0570-RA PubMed

  • [34]

    Gaggelli I, Scipioni F, Tirone A, Carli AF. Intra-abdominal desmoid tumors: a case report. Ann Ital Chir. 2014 Oct 7;85(ePub). pii: S2239253X14023056. PMID: 25335448 Google Scholar

  • [35]

    Mussi CE, Colombo P, Lo Russo C, Kasangian A, Cananzi F, Marrari A, et al .Sporadic desmoid tumors of the abdominal wall:the results of surgery. Tumori, 2016;102(6),582-587. doi: 10.5301/tj.5000552 Web of ScienceCrossrefGoogle Scholar

  • [36]

    Carlomagno N, Santangelo ML, Amato B, Calogero A, Saracco M, Cremone C, et al., Total colectomy for cancer, analysis of factors linked to patients’ age. International Journal of Surgery 2014, 12 (S2), S135-139 CrossrefWeb of ScienceGoogle Scholar

  • [37]

    Santangelo ML, Criscitiello C, Renda A, Federico S, Curigliano G, Dodaro C, et al. Immunosuppression and Multiple Primary Malignancies in Kidney-Transplanted Patients: A Single-Institute Study. Biomed Res Int. 2015;2015:183523.  CrossrefPubMedWeb of ScienceGoogle Scholar

  • [38]

    Koskenvuo, L, Pitkaniemi, J, Rantanen, M, Lepisto, A. Impact of screening on survival in familial adenomatous polyposis. J. Clin. Gastroenterol. 2016; 50: 40-44 CrossrefPubMedWeb of ScienceGoogle Scholar

  • [39]

    Lasseur A, Pasquer A, Feugier P, Poncet G. Sporadic intraab-dominal desmoid tumor: a unusual presentation. J Surg Case Rep. 2016, 2016(5), rjw070 CrossrefGoogle Scholar

  • [40]

    Vasen, HFA, Möslein G, Alonso A, Aretz S, Bernstein I, Bertario L A, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 2008; 57: 704-713 Web of SciencePubMedCrossrefGoogle Scholar

About the article


Received: 2019-02-22

Accepted: 2019-06-12

Published Online: 2019-07-19


Competing interests: The authors declare that they have no competing interests.

Funding Statement: No funding was received

Authors’ contributions: AC, CS, and NC equally contributed to this work, designed the study, and wrote the manuscript. All authors were involved in the study’s concept, data analysis of the manuscript, and the critical revision of the manuscript for important intellectual content. All authors revised and approved the final version of the article.


Citation Information: Open Medicine, Volume 14, Issue 1, Pages 572–576, ISSN (Online) 2391-5463, DOI: https://doi.org/10.1515/med-2019-0064.

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© 2019 Armando Calogero et al., published by De Gruyter. This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License. BY-NC-ND 4.0

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