Scientific Letter
Calcifying Cystic Fibrous Tumour. A Rare Form of Benign Peritoneal Carcinomatosis
Tumor fibroso quístico calcificante. Una extraña forma de carcinomatosis peritoneal benigna
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Una extraña forma de carcinomatosis peritoneal benigna" "tienePdf" => "es" "tieneTextoCompleto" => "es" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "338" "paginaFinal" => "339" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Calcifying cystic fibrous tumour. a rare form of benign peritoneal carcinomatosis" ] ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figura 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 715 "Ancho" => 950 "Tamanyo" => 316411 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Nódulos constituidos por haces de colágeno.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Manuel Bellver Oliver, Jorge 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"<span class="elsevierStyleTextfn">Scientific Letter</span>" "titulo" => "Calcifying Cystic Fibrous Tumour. A Rare Form of Benign Peritoneal Carcinomatosis" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "338" "paginaFinal" => "339" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Manuel Bellver Oliver, Jorge Arredondo Chaves, Pachi Queipo Gutierrez, Victor Valentí Azcárate, Fernando Rotellar Sastre" "autores" => array:5 [ 0 => array:4 [ "nombre" => "Manuel" "apellidos" => "Bellver Oliver" "email" => array:1 [ 0 => "mbellver@unav.es" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Jorge" "apellidos" => "Arredondo Chaves" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Pachi" "apellidos" => "Queipo Gutierrez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Victor" "apellidos" => "Valentí Azcárate" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "Fernando" "apellidos" => "Rotellar Sastre" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Departamento de Cirugía General y del Aparato Digestivo, Clínica Universidad de Navarra, Pamplona, Navarra, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Anatomía Patológica, Clínica Universidad de Navarra, Pamplona, Navarra, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tumor fibroso quístico calcificante. Una extraña forma de carcinomatosis peritoneal benigna" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 739 "Ancho" => 982 "Tamanyo" => 342829 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Nodules formed by collagen bundles.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">The calcifying cystic fibrous tumour (CCFT) is an extremely rare benign tumour first described in 1998 in children, as a <span class="elsevierStyleItalic">childhood fibrous tumour with psammoma bodies</span>.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Subsequently, in 1993 a series of 10 cases was described in patients between 1 and 33 years of age, and was named <span class="elsevierStyleItalic">calcifying fibrous pseudotumour</span>.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> At present, the WHO recommends calling it a tumour, due to the possibility of local recurrence.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 31-year-old woman with grade II obesity associated with arthritis, high percentage of body fat, glucose intolerance, hyperfibrinogenemia, hyperandrogenism and hyperleptinemia. During surgery–laparoscopic tubular gastrectomy–multiple implants were observed mainly in the greater omentum with a pearly appearance (between 1 and 4<span class="elsevierStyleHsp" style=""></span>mm) of hard consistency, and encapsulated, suggestive of peritoneal carcinomatosis. An intraoperative biopsy was performed and the result was inconclusive; although the signs were compatible with a benign process a peritoneal dissemination due to a digestive or gynaecological tumour could not be ruled out.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Before surgery the patient had had a gynaecological examination and a colonoscopy, and both had been normal. Furthermore, during evaluation for bariatric surgery, an upper endoscopy had been performed that was also normal.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The abdominal cavity, pelvis, Douglas cul-de-sac, both lower quadrants, ovaries, liver and spleen were carefully inspected, and no pathologic findings were found. Only one other mass of a larger size–1<span class="elsevierStyleHsp" style=""></span>cm–but of similar characteristics, was found close to the cecal appendix. Because of the uncertainty of diagnosis, the bariatric procedure was postponed, and omentectomy and appendectomy were performed with a therapeutic intent, as the appendix was considered as the possible primary tumour, in order to obtain an R0 resection and await the definitive pathological diagnosis.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The pathology study showed nodules formed by thick collagen bundles with hyalinised areas, some of them calcified with psammmoma bodies. A vascular network of fine walls with an inflammatory infiltrate surrounding it predominantly of plasmocytes and occasional lymphocyte was observed. The nodules were well delimited from the fibroadipose tissue and covered by a layer of mesothelium with focal hyperplasia in small nests (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). An immunohistochemical analysis was performed for antibodies against CD-32, CD-34, Keratin AE3/AE1, cytokeratin 5/6 and calretinin, and a diagnosis of multiple peritoneal CCFT was made.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">CCFT is an exceptionally rare benign tumour; to date there have only been 70 cases reported and the published series are very small.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> It is considered that 90% are solitary tumours located in soft tissues: suprarenal glands, spleen, liver, stomach, spermatic chord, pleura, peritoneum or omentum.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> Only 10% of CCFT are multiple and there are only 3 cases described of multiple abdominal CCFT,<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5–7</span></a> 4 if we add the present case, which would also be the first multiple peritoneal CCFT described in the Spanish literature. The clinical presentation of CCFT is variable, from an asymptomatic mass or incidental finding at surgery or autopsy to abdominal pain similar to an acute appendicitis in the second or third decade of life, with no difference between the sexes.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4–6,8,9</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The aetiology of this tumour is unknown although some authors have related it to chronic inflammatory states, trauma, prior surgery or a late sclerosing stage of an inflammatory myofibroblastic tumour. There are also theories about a certain genetic base and there is a case described of familial presentation.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5,6,10</span></a> Image diagnosis is performed using CT scan, as plain X-rays and ultrasound have a low efficiency.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The definitive diagnosis is exclusively pathological. Macroscopically they present as well circumscribed tumours in soft tissue areas that can vary in size from a few millimetres to 25<span class="elsevierStyleHsp" style=""></span>cm, and can be solitary or multiple.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,9</span></a> Microscopically, they present nests of fibrous tissue and hyalinized collagen, with scarce cellularity, and psammoma bodies.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3–7,9,10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The immunohistochemical studies reveal a positive reaction to vimentin, actin 1 A4, and variable to CD 34, and are negative for ALK.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,9,10</span></a> Differential diagnosis should be made with a peritoneal carcinomatosis, inflammatory myofibrobastic tumour, nodular fasciculitis, fibromatosis, aponeurotic calcifying fibroma or amyloidoma.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9,10</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Definitive treatment is surgical resection of the lesion or lesions and a laparoscopic approach is valid, although it has been performed in only two cases including this one.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,9,10</span></a> Recurrence is rare and no metastatic spread has been described, therefore an extensive follow-up does not seem necessary.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,6,7,9</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Bellver Oliver M, Arredondo Chaves J, Queipo Gutierrez P, Valentí Azcárate V, Rotellar Sastre F. Tumor fibroso quístico calcificante. Una extraña forma de carcinomatosis peritoneal benigna. Cir Esp. 2013;91:338–339.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 739 "Ancho" => 982 "Tamanyo" => 342829 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Nodules formed by collagen bundles.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Childhood fibrous tumor with psammoma bodies. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 3 | 0 | 3 |
| 2024 October | 16 | 5 | 21 |
| 2024 September | 23 | 14 | 37 |
| 2024 August | 15 | 11 | 26 |
| 2024 July | 26 | 11 | 37 |
| 2024 June | 24 | 4 | 28 |
| 2024 May | 35 | 6 | 41 |
| 2024 April | 24 | 9 | 33 |
| 2024 March | 18 | 4 | 22 |
| 2024 February | 16 | 2 | 18 |
| 2024 January | 25 | 1 | 26 |
| 2023 December | 25 | 4 | 29 |
| 2023 November | 22 | 7 | 29 |
| 2023 October | 15 | 7 | 22 |
| 2023 September | 20 | 3 | 23 |
| 2023 August | 20 | 4 | 24 |
| 2023 July | 16 | 4 | 20 |
| 2023 June | 28 | 5 | 33 |
| 2023 May | 26 | 2 | 28 |
| 2023 April | 44 | 4 | 48 |
| 2023 March | 25 | 3 | 28 |
| 2023 February | 30 | 2 | 32 |
| 2023 January | 37 | 6 | 43 |
| 2022 December | 37 | 6 | 43 |
| 2022 November | 34 | 10 | 44 |
| 2022 October | 32 | 5 | 37 |
| 2022 September | 37 | 17 | 54 |
| 2022 August | 32 | 11 | 43 |
| 2022 July | 31 | 7 | 38 |
| 2022 June | 23 | 7 | 30 |
| 2022 May | 24 | 8 | 32 |
| 2022 April | 56 | 7 | 63 |
| 2022 March | 65 | 7 | 72 |
| 2022 February | 78 | 5 | 83 |
| 2022 January | 55 | 7 | 62 |
| 2021 December | 32 | 7 | 39 |
| 2021 November | 69 | 13 | 82 |
| 2021 October | 46 | 14 | 60 |
| 2021 September | 37 | 11 | 48 |
| 2021 August | 32 | 5 | 37 |
| 2021 July | 28 | 11 | 39 |
| 2021 June | 19 | 7 | 26 |
| 2021 May | 24 | 12 | 36 |
| 2021 April | 31 | 19 | 50 |
| 2021 March | 46 | 3 | 49 |
| 2021 February | 20 | 8 | 28 |
| 2021 January | 17 | 10 | 27 |
| 2020 December | 17 | 6 | 23 |
| 2020 November | 16 | 6 | 22 |
| 2020 October | 17 | 5 | 22 |
| 2020 September | 27 | 12 | 39 |
| 2020 August | 14 | 9 | 23 |
| 2020 July | 18 | 13 | 31 |
| 2020 June | 24 | 13 | 37 |
| 2020 May | 16 | 21 | 37 |
| 2020 April | 20 | 6 | 26 |
| 2020 March | 20 | 5 | 25 |
| 2020 February | 19 | 7 | 26 |
| 2020 January | 22 | 13 | 35 |
| 2019 December | 16 | 11 | 27 |
| 2019 November | 14 | 9 | 23 |
| 2019 October | 11 | 5 | 16 |
| 2019 September | 28 | 4 | 32 |
| 2019 August | 15 | 0 | 15 |
| 2019 July | 31 | 19 | 50 |
| 2019 June | 45 | 25 | 70 |
| 2019 May | 135 | 42 | 177 |
| 2019 April | 44 | 21 | 65 |
| 2019 March | 6 | 7 | 13 |
| 2019 February | 9 | 10 | 19 |
| 2019 January | 12 | 6 | 18 |
| 2018 December | 6 | 10 | 16 |
| 2018 November | 11 | 4 | 15 |
| 2018 October | 14 | 5 | 19 |
| 2018 September | 10 | 2 | 12 |
| 2018 August | 3 | 4 | 7 |
| 2018 July | 6 | 7 | 13 |
| 2018 June | 2 | 0 | 2 |
| 2018 May | 5 | 10 | 15 |
| 2018 April | 11 | 0 | 11 |
| 2018 March | 10 | 1 | 11 |
| 2018 February | 6 | 3 | 9 |
| 2018 January | 5 | 1 | 6 |
| 2017 December | 6 | 1 | 7 |
| 2017 November | 10 | 4 | 14 |
| 2017 October | 8 | 13 | 21 |
| 2017 September | 6 | 4 | 10 |
| 2017 August | 19 | 16 | 35 |
| 2017 July | 9 | 2 | 11 |
| 2017 June | 13 | 15 | 28 |
| 2017 May | 15 | 6 | 21 |
| 2017 April | 18 | 3 | 21 |
| 2017 March | 20 | 20 | 40 |
| 2017 February | 10 | 3 | 13 |
| 2017 January | 4 | 0 | 4 |
| 2016 December | 8 | 4 | 12 |
| 2016 November | 11 | 4 | 15 |
| 2016 October | 21 | 5 | 26 |
| 2016 September | 27 | 9 | 36 |
| 2016 August | 12 | 4 | 16 |
| 2016 July | 11 | 4 | 15 |
| 2016 June | 17 | 8 | 25 |
| 2016 May | 15 | 20 | 35 |
| 2016 April | 18 | 22 | 40 |
| 2016 March | 15 | 29 | 44 |
| 2016 February | 11 | 22 | 33 |
| 2016 January | 6 | 21 | 27 |
| 2015 December | 14 | 12 | 26 |
| 2015 November | 11 | 16 | 27 |
| 2015 October | 22 | 12 | 34 |
| 2015 September | 25 | 6 | 31 |
| 2015 August | 17 | 2 | 19 |
| 2015 July | 10 | 3 | 13 |
| 2015 June | 5 | 1 | 6 |
| 2015 May | 9 | 3 | 12 |
| 2015 April | 12 | 6 | 18 |
| 2015 March | 16 | 5 | 21 |
| 2015 February | 10 | 3 | 13 |
| 2015 January | 19 | 3 | 22 |
| 2014 December | 40 | 9 | 49 |
| 2014 November | 18 | 3 | 21 |
| 2014 October | 21 | 5 | 26 |
| 2014 September | 19 | 2 | 21 |
| 2014 August | 21 | 1 | 22 |
| 2014 July | 14 | 3 | 17 |
| 2014 June | 9 | 2 | 11 |
| 2014 May | 15 | 2 | 17 |
| 2014 April | 11 | 0 | 11 |
| 2014 March | 5 | 2 | 7 |
| 2014 February | 13 | 6 | 19 |
| 2014 January | 11 | 9 | 20 |
| 2013 December | 16 | 3 | 19 |
| 2013 November | 3 | 3 | 6 |
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