Peripheric primitive neuroectodermal tumor (Askin)

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Abstract

The case of preoperative diagnosis of peripheric primitive neuroectodermal tumor (pPNET) of the chest with damage of body sternum in 21-year-old patient is presented in Native literature for the first time. CT and echography data as well as serum tumor-markers level are given. The detection of tissue-markers and electronic microscopy data are the most reliable diagnostic methods. However clinical and routine morphologic examinations are important as well. Our patient received neoadjuvant chemotherapy followed by chest resection and plasty by osseous-musculocutaneous flap on vascular pedicle. Efficacy of polychemotherapy with vincristine, cyclophosphan, farmorubicin and platidiam is proved. Nine months after operation no tumor relapse and metastasis were observe. In spite of relatively low sensitivity of pPNET to cytostatic drugs, combined treatment with neoadjuvant is the most justified. Original method for the plasty of chest defect with no application of artificial materials provides favourable conditions for wound healing with good and satisfactory cosmetic outcomes.

About the authors

A. N. Makhson

Moscow City Clinical Oncology Hospital No. 62

Author for correspondence.
Email: info@eco-vector.com
Russian Federation, Moscow

A. S. Burlakov

Moscow City Clinical Oncology Hospital No. 62

Email: info@eco-vector.com
Russian Federation, Moscow

I. V. Kuzmin

Moscow City Clinical Oncology Hospital No. 62

Email: info@eco-vector.com
Russian Federation, Moscow

K. K. Pugachev

Moscow City Clinical Oncology Hospital No. 62

Email: info@eco-vector.com
Russian Federation, Moscow

M. I. Popov

Moscow City Clinical Oncology Hospital No. 62

Email: info@eco-vector.com
Russian Federation, Moscow

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Supplementary files

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2. Fig. 1. Computed tomogram. Volumetric formation 9 × 7.5 × 6.6 cm in size with a heterogeneous structure, density +50 units, infiltrating the sternum (destruction of the lower third of its body at the level of the VIII thoracic vertebra). The tumor invades the subcutaneous tissue and the anterior mediastinum, intimately adjacent to the anterior sections of the heart.Rice. 1. Computed tomogram. Volumetric formation 9 × 7.5 × 6.6 cm in size with a heterogeneous structure, density +50 units, infiltrating the sternum (destruction of the lower third of its body at the level of the VIII thoracic vertebra). The tumor invades the subcutaneous tissue and the anterior mediastinum, intimately adjacent to the anterior sections of the heart.

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3. Fig. 2 Aspiration biopsy of the tumor of the sternum with a thin needle. The cytogram is represented by small rounded malignant cells against the background of erythrocytes. Romanovsky-Giemsa staining. SW. 630.

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4. Fig. 3. Open biopsy of a tumor of the sternum. Small rounded cells are locally grouped into rosette structures. Stained with hematoxylin and eosin. SW. 100 (a) and 400 (b).

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5. Fig. 4. Mobilized pedunculated muscle flap with split rib fragments at the edge of the chest wall defect.

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6. Fig. 5. Three-dimensional computer reconstruction of a postoperative defect of the sternum and ribs. The upper displaced rib is visible, covering the defect of the sternum.

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7. Fig. 6. Operating drug. Crossed segments of the ribs and a leaf of the parietal pleura are visible.

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8. Fig. 7. Micropreparation of the removed tumor. Tumor tissue with signs of drug pathomorphosis: foci of necrosis, ugly forms of malignant cells with signs of pronounced dystrophy. Stained with hematoxylin and eosin. SW. 400.

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9. Fig. 8. Patient B. after resection of the chest wall with defect plasty with split rib fragments in a block with displaced scalene and latissimus muscle flaps.

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