A clinical case of sacrum and coccyx chordoma having a massive intrapelvic component (surgical treatment with a brief review of the literature)

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Abstract

BACKGROUND: Chordoma is a rare malignant tumor that develops from the remnants of the notochord and is located in the axial skeleton in the absolute majority of cases. It is most often localized in the sacrum, coccyx and pelvis, and is characterized by an initially asymptomatic long-time course, making early diagnosis difficult. Radical surgical treatment is the leading factor allowing to prolong the recurrence-free and overall survival of patients with chordoma, but it is often difficult due to complex anatomical localization of the tumor, as well as delayed medical treatment, frequently accompanied with the subsequent development of neurological complications, while in elderly patients with high comorbidity it is not always feasible.

CLINICAL CASE DESCRIPTION: We present a clinical case of radical surgical treatment of a patient with a S4-5 vertebral and coccyx chordoma showing a massive intrapelvic component. Clinical manifestations of the disease in the form of pain syndrome and pelvic organ dysfunction developed only when the tumor reached a larger size, forming a massive intrapelvic component up to 20 cm in size. The examination, including computer and magnetic resonance tomography, trepan biopsy with pathomorphologic examination, allowed to establish the diagnosis. Taking into account the size and localization of the tumor, the multidisciplinary team performed radical surgical intervention including sacral resection at the S3 level, coccygectomy with tumor removal. Morphological study of the removed tumor confirmed the diagnosis. In the early postoperative period, the wound healed by primary tension, the development of pelvic organs dysfunction was noted, which partially regressed by discharge. The article presents a brief review of the current problems of diagnosis and treatment of patients with chordoma.

CONCLUSION: Diagnosis and treatment of sacral chordoma is one of the most difficult problems of orthopedic oncology. A full preoperative examination and a multidisciplinary approach in this case made it possible to perform radical surgical intervention, reduce the risks of tumor progression, intra- and postoperative complications, and preserve the patient’s quality of life as much as possible.

About the authors

Anton G. Nazarenko

Priorov National Medical Research Center for Traumatology and Orthopedics

Email: nazarenkoag@cito-priorov.ru
ORCID iD: 0000-0003-1314-2887
SPIN-code: 1402-5186

MD, Dr. Sci. (Med.), Professor of the RAS

Russian Federation, Moscow

Vadim Yu. Karpenko

Priorov National Medical Research Center for Traumatology and Orthopedics

Email: Karpenko@cito-priorov.ru
ORCID iD: 0000-0002-8280-8163
SPIN-code: 1360-8298

MD, Dr. Sci. (Med.)

Russian Federation, Moscow

Aleksandr F. Kolondaev

Priorov National Medical Research Center for Traumatology and Orthopedics

Email: klnd@inbox.ru
ORCID iD: 0000-0002-4216-8800
SPIN-code: 5388-2606

MD, Cand. Sci. (Med.)

Russian Federation, Moscow

Nikita A. Lyubeznov

Priorov National Medical Research Center for Traumatology and Orthopedics

Email: nikitkalyubeznov@gmail.com
Russian Federation, Moscow

Gennadiy N. Berchenko

Priorov National Medical Research Center for Traumatology and Orthopedics

Email: berchenko@cito-bone.ru
ORCID iD: 0000-0002-7920-0552
SPIN-code: 3367-2493

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Igor N. Karpov

Priorov National Medical Research Center for Traumatology and Orthopedics

Author for correspondence.
Email: igoukarpoff@mail.ru
ORCID iD: 0000-0002-3135-9361
SPIN-code: 5943-3689

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Mikhail V. Alekseev

Ryzhikh National Medical Research Centre for Coloproctology

Email: doctor-pro@mail.ru
ORCID iD: 0000-0001-5655-6567
SPIN-code: 9343-8872

MD, Dr. Sci. (Med.)

Russian Federation, Moscow

Aleksandr M. Kuzminov

Ryzhikh National Medical Research Centre for Coloproctology

Email: info@gnck.ru
ORCID iD: 0000-0002-7544-4752
SPIN-code: 4255-0201

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Yuliya V. Alimova

Ryzhikh National Medical Research Centre for Coloproctology

Email: doctoralimova@gmail.com
ORCID iD: 0000-0001-7245-4042
SPIN-code: 1828-7903
Russian Federation, Moscow

Anatoly L. Karasev

Priorov National Medical Research Center for Traumatology and Orthopedics

Email: karaseva81@mail.ru
ORCID iD: 0000-0002-3356-5193
Russian Federation, Moscow

Kirill A. Antonov

Priorov National Medical Research Center for Traumatology and Orthopedics

Email: osteopathology6@mail.ru
Russian Federation, Moscow

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Supplementary files

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1. JATS XML
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2. Fig. 1. T2 SPAIR coronal MRI showing a heterogeneously hyperintense tumor occupying a significant portion of the pelvic cavity (asterisk). Pronounced heterogeneity of the signal is due to areas of hemorrhage

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3. Fig. 2. MRI in T1 SPIR mode in the sagittal plane: a significant size, heterogeneously hypointense, with moderately hyperintense areas, the intrapelvic soft tissue component of the tumor (asterisk) is adjacent to the anterior surface of the sacrum, infiltrating muscle tissue in the posteroinferior direction (arrow)

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4. Fig. 3. CT in the sagittal plane, there is a lytic focus of destruction of the C4, C5 and coccygeal vertebrae (arrow), a massive soft tissue component in the pelvic cavity, reaching the level of the L5 vertebra (asterisk)

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5. Fig. 4. Anterior approach stage, colon exposure

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6. Fig. 5. Posterior approach to the sacrum and coccyx. A wide resection is performed within healthy soft tissues

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7. Fig. 6. Posterior view of the wound after tumor removal. The image visualizes the edge of the resection of the sacrum at the S3 level (asterisk), rectum (arrow)

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8. Fig. 7. Photograph of a gross specimen of a tumor removed en bloc with its covering tissues

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9. Fig. 8. Three-dimensional CT reconstruction after surgery, posterior view. Resection of the sacrum at the level of the S3 vertebra (arrow)

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10. Fig. 9. Photograph of a macroscopic specimen: longitudinal section of a fragment of the sacrum and a tumor node growing from it. Numerous areas of hemorrhage are noteworthy

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11. Fig. 10. Micrograph. Conventional chordoma with characteristic physaliform cells with pronounced vacuolization of the light cytoplasm. Hematoxylin and eosin staining. ×400

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12. Fig. 11. Micrograph. Conventional chordoma. An area of densely packed epithelial-like cells with mild vacuolation and eosinophilic cytoplasm. Hematoxylin and eosin staining. ×400

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