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Malignant Tumours

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Book cover Coloproctology

Part of the book series: European Manual of Medicine ((EUROMANUAL))

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Abstract

9.1

The two main inherited colorectal cancer syndromes, familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC) or Lynch syndrome are characterised by a single mutation leading to a dramatically increased predisposition for colorectal cancer. FAP is an autosomal dominantly inherited condition that has been shown to be due to mutations in the adenomatous polyposis coli (APC) gene, a tumour suppressor gene active in the Wnt/Wingless signalling pathway. The much commoner HNPCC is caused by a germline mutation in one of the DNA mismatch repair genes (hMLH1, hMSH2, hMSH6). These genes correct errors of DNA replication and any defect of this repair system will lead to rapid accumulation of mutations. Both syndromes are characterised by early onset of colorectal tumours, by synchronous and metachronous tumours as well as numerous extracolonic benign and malignant manifestations.

9.2

Colon cancer is common and usually presents with a history of altered bowel habit, rectal bleeding or anaemia. The onset and severity of symptoms depends on tumour location. Advanced disease at first presentation is not uncommon, as diagnosis of proximal tumours is difficult and often delayed. Outcome is most closely related to the extent of disease at presentation. Surgical resection is the primary treatment for any, also advanced stages; adjuvant chemotherapy improves outcome.

9.3

The main treatment in rectal cancer is surgery. An ongoing debate is whether a local excision or an abdominal procedure should be done. Also the use of radiotherapy given pre- or postoperatively has been explored in many trials. Based upon all trials, if radiotherapy should be used, it should be given preoperatively. A third debate is the use of chemotherapy. Although evidence is not as strong as the use of radiotherapy, most units are following the same principles as are used for colon cancer. The whole decision making on how to treat the patients with local excision or abdominal surgery or adding radiotherapy and/or chemotherapy must be based upon preoperative staging, where endoanal ultrasound (early lesion) and MRI (advanced cases) is mandatory. The gold standard regarding decision-making of preferable treatment for patients with a rectal cancer is to be judged preoperatively in a multidisciplinary team (MDT) conference as well as after surgery when the pathological report should be evaluated.

9.4

Anal cancer is rare and may be confused with benign proctological conditions, leading to delayed presentation and diagnosis. Treatment has changed radically since the late 1980s from primarily radical surgical excision to combined chemoradiotherapy with surgery generally reserved for persistent or recurrent disease.

9.5

The international classification of malignant tumours of any site is based on four axes: 1) topography; 2) histomorphology (typing and grading); 3) anatomical extent before treatment (clinical: TNM or cTNM, pathological: pTNM, stage grouping); 4) anatomical extent after treatment (residual tumour or R classification). The classification of histomorphology and anatomical extent before treatment are site-specific for anal canal cancer, perianal (anal margin) cancer and rectal cancer. In some defined situations, additional classifications are necessary: 1) after neoadjuvant treatment: histological regression grading; 2) after rectal cancer surgery with total or partial mesorectal excision: pathological assessment of the quality of mesorectal excision; 3) after abdominoperineal excision: pathological assessment of the quality of this operation.

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Chapter 9.1

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Author information

Authors and Affiliations

  1. Hôpital Saint-Antoine, AP-HP - Department of Surgery, Université Pierre et Marie Curie, 184 rue du Faubourg Saint-Antoine, 75012, Paris, France

    Malika Bennis

  2. Service de chirurgie générale et digestive, Hôpital St. Antoine, 184 rue du Faubourg Saint-Antoine, 75 012, Paris, France

    Emmanuel Tiret

  3. Chirurgische Klinik, Universitätsklinikum Erlangen-Nürnberg, Krankenhausstraße 12, 91054, Erlangen, Germany

    Klaus E. Matzel, Wei Zhang & Paul Hermanek

  4. Department of Surgery, University Hospital Uppsala, 751 85, Uppsala, Sweden

    Lars Påhlman

  5. St. Vincent’s University Hospital, University College Dublin, Elm Park, Dublin 4, Dublin, Ireland

    P. Ronan O’Connell

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  1. Malika Bennis
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  2. Emmanuel Tiret
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  3. Klaus E. Matzel
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  4. Wei Zhang
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  6. P. Ronan O’Connell
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Editor information

Editors and Affiliations

  1. Enddarm-Zentrum Mannheim, Bismarckplatz 1, 68165, Mannheim, Germany

    Alexander Herold

  2. Institut des Maladies de l’Appareil Digestif, Clinique Chirurgicale, Hôtel-Dieu, Place Ricordeau, 44093, Nantes Cedex, France

    Paul-Antoine Lehur

  3. Chirurgische Klinik, Universitätsklinikum Erlangen-Nürnberg, Krankenhausstraße 12, 91054, Erlangen, Germany

    Klaus E. Matzel

  4. University College Dublin, St. Vincent’s University Hospital, Elm Park, Dublin, Ireland

    P. Ronan O'Connell

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Bennis, M. et al. (2008). Malignant Tumours. In: Herold, A., Lehur, PA., Matzel, K., O'Connell, P. (eds) Coloproctology. European Manual of Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-71217-6_9

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  • DOI: https://doi.org/10.1007/978-3-540-71217-6_9

  • Publisher Name: Springer, Berlin, Heidelberg

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