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Cutaneous lymphangitis carcinomatosa in salivary duct carcinoma
  1. Maike JM Uijen1,
  2. Jetty AM Weijers1,
  3. Gerben Lassche1,
  4. Stefan G van Ravensteijn1,
  5. Maartje C van Rijk2,
  6. Satish FK Lubeek3,
  7. Adriana CH van Engen-van Grunsven4,
  8. Avital Amir4,
  9. Chantal ML Driessen1,
  10. Carla ML van Herpen1
  1. 1 Medical Oncology, Radboudumc, Nijmegen, The Netherlands
  2. 2 Medical Imaging, Radboudumc, Nijmegen, The Netherlands
  3. 3 Dermatology, Radboudumc, Nijmegen, The Netherlands
  4. 4 Pathology, Radboudumc, Nijmegen, The Netherlands
  1. Correspondence to Carla ML van Herpen, Radboudumc, 6595 GA Nijmegen, Gelderland, The Netherlands; carla.vanherpen{at}

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Salivary duct carcinoma (SDC) is one of the most aggressive subtypes of salivary gland cancer. It is a very rare cancer (incidence 0.1–0.2 per 100 000 people/year), characterised by androgen receptor (AR) expression in almost all patients and HER2 expression in approximately 30% of patients.1

A typical characteristic of SDC is the high propensity for lymph node metastases, with a median number of tumour positive lymph nodes of 4 (range: 0–97).2 There is a tendency towards worse survival when more lymph nodes are involved.2 Here, we present cutaneous lymphangitis carcinomatosa (CLC) as a relatively frequently occurring manifestation of advanced SDC. With this report, we aim to increase the knowledge on this disease manifestation, because it is sometimes not recognised, although it is typical for this subtype of salivary gland cancer.

In our tertiary referral centre, 11 SDC patients developed skin lesions in the neck and chest region. All patients had extensive lymph node metastases at diagnosis (table 1). Skin biopsies showed CLC and cutaneous metastases (figures 1 and 2). Two representative cases will be described in detail. Furthermore, we report on the molecular analysis of these 11 patients (online supplemental table 1).

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Table 1

Patient characteristics of SDC patients at time of diagnosis of CLC

Figure 1

Clinical and pathology images of case 1. (A) Clinical image of case 1 at the first presentation of CLC, showing a non-scaling erythematous plaque with variable induration on the left side of the neck and chest. (B) Pathological evaluation of the skin biopsy revealed tumour cells within the lumina of dermal lymphatic vessels and infiltration into the dermis and hypodermis (D2-40 staining ×330, staining for lymphatic vessels; pan-cytokeratin staining CK AE 1/3 …

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  • MJU and JAW are joint first authors.

  • Handling editor Runjan Chetty.

  • MJU and JAW contributed equally.

  • Contributors Study concepts: MU, JW, CvH. Study design: MU, JW, GL, SvR, MvR, SL, IvE-VG, AA, CD, CvH. Data acquisition: MU, JW, GL, SvR, MvR, SL, IvE-VG, AA, CD, CvH. Data analysis and interpretation: MU, JW, GL, SvR, MvR, SL, IvE-VG, AA, CD, CvH. Manuscript preparation: MU, JW, GL. Manuscript editing: MU, JW, GL, SvR, MvR, SL, IvE-VG, AA, CD, CvH. Manuscript review: MU, JW, GL, SvR, MvR, SL, IvE-VG, AA, CD, CvH.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.