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A giant intraperineal and extraperineal mass
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  1. Giancarlo D'Andrea1,
  2. Pietro Familiari1,
  3. Laura Lorenzon2,
  4. Giuseppe Argento3,
  5. Antonino Raco1,
  6. Vincenzo Ziparo2
  1. 1Division of Neurosurgery, Department of Neurosciences, Organ Senses and Mental Health, Faculty of Medicine and Psychology, University of Rome “La Sapienza”, Rome, Italy
  2. 2Surgery 1 Unit, Surgical and Medical Department of Traslational Medicine, Faculty of Medicine and Psychology, University of Rome “La Sapienza”, Rome, Italy
  3. 3Radiology Unit, Surgical and Medical Department of Traslational Medicine, Faculty of Medicine and Psychology, University of Rome “La Sapienza”, Rome, Italy
  1. Correspondence to Professor Laura Lorenzon, laura.lorenzon{at}uniroma1.it

https://doi.org/10.1136/bcr-2014-206711

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    Description

    A 67-year-old woman was admitted to our hospital with severe low back/radicular pain and gait disturbance, and presenting a giant extraperineal mass (figure 1).

    Figure 1
    Figure 1

    (A) Clinical appearance of the perineal mass protruding outside the perineum in the infragluteal space (‘queen bee’ shape). (B) Preoperative iodine contrast-enhanced CT scan documenting the tumour: (B1 and B2) CT sagittal and coronal multiplanar rendering views: in the CT reformation images both intrapelvic and extraperineal components of the mass are shown; CT axial images: (B3) pelvic involvement and superior aspect of the extraperineal mass; (B4) axial plane at the level of the proximal third of the thighs documenting the maximum diameter of the extraperineal component. (C) MRI: turbo spin-echo T2-weighted axial images at different levels; (C1 and C2) intrapelvic extension of the mass in the retroperitoneal space and in the posterior sacral region; (C3 and C4) perineal and intraperineal major cystic component close to the left para-sagittal plain, adjacent to the proximal left thigh. (D) Posterior surgical approach to the tumour.

    A preoperative CT scan documented a mixed fluid/solid mass measuring 35×19×18 cm dislocating the rectum and protruding outside the perineum in the infragluteal space, which was also associated with a sacrococcygeal malformation consistent with a coccyx's cleft, as confirmed by MRI.

    The patient reported the presence of a smaller sacrococcygeal mass since childhood, which increased in size over the past 3 years; she did not consider a medical consultation, however, due to a major depressive disorder.

    The patient was scheduled for a surgical procedure and the tumour was completely removed via a posterior approach. The pathological examination documented a teratoma with focal areas of malignant transformation.

    Sacrococcygeal teratomas (SCTs) are germ cell tumours, with an incidence of 1/40 000 live-births, affecting females 4 times more often than males.1 ,2

    SCTs are the most common presacral germ cell tumours in children and neonates. Up to 27% of SCTs are malignant, and the probability of malignancy increases with the age of presentation.1

    SCTs are classified according to Altman classification into: type I, predominantly external masses with a small presacral component; type II, external masses with a significant intrapelvic component; type III, external masses with a pelvic and abdominal component; and type IV, internal masses with an intrapelvic and abdominal location; types II and III are dumbbell shaped.3

    Learning points

    • Because the clinical manifestations of presacral masses are often non-specific, imaging plays an important role in the detection and differentiation of these masses and might be crucial for their surgical management.

    • Sacro-coccygeal teratomas affect females more often than males.

    • Up to one-fourth of the SCT might be malignant and the risk is increased in adult patients.

    References

    1. ↵
      1. Peterson CM,
      2. Buckley C,
      3. Holley S, et al
      . Teratomas: a multimodality review. Curr Probl Diagn Radiol 2012;41:210–19.
      OpenUrlCrossRefPubMed
    2. ↵
      1. Kocaoglu M,
      2. Frush DP
      . Pediatric presacral masses. RadioGraphics 2006;26:833–57.
      OpenUrlCrossRefPubMedWeb of Science
    3. ↵
      1. Altman RP,
      2. Randolph JG,
      3. Lilly JR
      . Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey—1973. J Pediatr Surg 1974;9:389–98.
      OpenUrlCrossRefPubMedWeb of Science
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    Footnotes

    • Contributors All authors contributed to writing the manuscript; and reviewed and approved the manuscript in its final form.

    • Competing interests None.

    • Patient consent Obtained.

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