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About Ivo and His Sarcoma Story

How everything started

Sarcoma patient story

In the summer of 2020, Ivo began to feel the need for more rest. In the mornings when he woke up, his lower back hurt, but he has always had minor problems with his lower back. The pain went away during the day. He started having occasional sharp abdominal pain. He first visited a doctor for his lower back, but after tests, they found that he had a slight sprain. He started treatment and painkillers, which did not help.

After that, we started looking for a doctor for his stomach pain. Unfortunately, we visited three gastroenterologists, who did not do a detailed examination with an echographia and only prescribed drugs based on the symptoms.

We lost valuable time (nearly a month). When we found a doctor to carry out a detailed examination with ultrasound, it turned out that Ivo had a 15-centimetre tumour in the peritoneum, metastases in the liver and he was full of ascites. We are based in Bulgaria and the doctor told us that if he was to have any chance, we must look for treatment in Turkey, Israel or the USA. His idea was that these countries have more aggressive treatment plans.

The Treatment:

Medical documentation

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Ivo applied to Anadolu Hospital in 10/2020 due to the tumor in left retroperitoneal localization and with peritoneal extension in the abdomen.

High grade small round cell carcinoma was diagnosed subsequent to the pathology consultation. ES markers (-).

Systemic chemotherapy (Vac/IE) was started by medical oncology dept. PET/CT (4/2021): Marked regression. Systemic therapy was continued and a total of 11 courses have been administered.

Surgery:

Medical Documentation

The patient was evaluated considering the information that he has the diagnosis of small cell sarcoma and has been followed up / treated for intraabdominal diffuse involvement.

Immunophenotypic and molecular pathological findings of the previous biopsy of the same localization, which was consulted to our department (No: AMC B-5477-2020) were considered during the analysis. Desmin and WT1 were found to be diffuse positive while PanCK and SMA were found to be diffuse positive in patchy form in tumor cells in the repeated immunohistochemical analysis. LCA, S100, melanoma cocktail, NKX2.2, SS18-SSX, MdM2, Dog1 were found to be negative. Diffuse positive SatB2 was found in lateral to the tumor, which may be artificial. Ki-67 proliferation index is 80%. Mature-like chondroid areas were seen in the tumor.

Desmoplastic small round cell tumor (DSRCT), CIC rearranged sarcoma, small cell osteosarcoma, dedifferentiated chondrosarcoma, dedifferentiated liposarcoma, MPNST, rhabdomyosarcoma, Ewing tumor, synovial sarcoma, small cell undifferentiated sarcoma, poor differentiate / undifferentiated carcinoma and lymphoma were included in the differential diagnosis. Although immunophenotypic findings primarily suggest desmoplastic small round cell tumor (DSRCT), no evidence of specific translocation was found in the analysis of the previous biopsy. Differentiation for CIC rearranged sarcoma is through more advanced molecular analyses.

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  • The actual procedure: Robotic gross total tumor excision.
  • Tumor localization: Left Retroperitoneum
  • Тumor depth (NCCN-STS): Deep.
  • Tumor margins (NCCN-STS): Well-circumscribed.
  • Tumor dimensions: 14x10x8 cm.
  • Histological type (WHO Classification): Histological grade (French Federation of Cancer Centers Sarcoma Group (FNCLCC): Grade 3
  • TNM grading: High grade.
  • Hypercellularity: High
  • Mitotic activity: >20 /1 mm2
  • Presence necrosis: Present (5%)
  • Condition of Surgical Margins: Macroscopic tumor continuity in the surgical margins.
  • Additional findings: Presence of inflammatory infiltrates: None
  • Lymphovascular invasion: None
  • Efficacy of the therapy was determined: Necrosis, fibrosis
  • Viable tumor percentage: >90%
  • The status of regional lymph nodes: No lymph node in the specimens. A tumoral implant was seen in the parietal peritoneum.
  • Macroscopy Perietal peritoneum, tumor-suspected lesion, biopsy: Solid nodular tissues measuring up to 0.5 cm were sampled (FS)
  • Retroperitoneal mass: A soft tissue mass with lobulated contour and dimensions of 14x10x8 cm. Hemorrhage and cystic areas were seen in its sections. Partially solid calcific areas were seen. Partial lipomatous areas in bright yellow color were noted. FS + partially 8 cassettes + 9 HE slides. SD/HB

Post-Operative Radiotherapy

Medical Documentation 

Surgery was indicated to the primary disease. Robotic tumor excision was performed on left retroperitoneal tumor on 15.7.2021. Pathology: Viable tumor in dimensions of 14x10x8 cm, grade III, necrosis 5%, surgical margin (+) [diffuse], LVI (-), no LN, tumoral implant is seen in parietal peritoneum. Post-operative radiotherapy is indicated due to its surgical margin positivity.

  • Physical Examination: kps:90
  • Histopathological diagnosis: High grade small round cell sarcoma
  • Diagnosis: Retroperitoneal tumor
  • Consumables List: Not used
  • Aim of Radiotherapy: curative
  • Treatment Device: Varian Edge Linear Accelerator
  • Energies Utilized: 6 MV
  • Fraction Count: 28 fractions
  • Fraction Doses: 2
  • Total Radiation Dose: 56 Gy
  • Total Area Count: Double arch

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Rad. Area and Technique: Following immobilization, planning CT and 4D CT for specifying tumor movements with
respiration are performed. Images obtained are analyzed together with pre-operative images to specify target and risky tissues. Using volumetric IMRT technique, plan to treat surgical site was made. Patient was treated with this plan in 28 fractions. IGRT is performed with CBCT before each fraction. Surface monitoring was done (vision RT OSMS) during the course of the entire treatment.

Concomitant Treatment: None
Rad. Start-Finish Date: 17.08.2021 – 23.09.2021
Clinical Follow-up: Abdominal bloating and pain started to develop during the treatment. In the subsequent radiologic examination, progression was observed. Radiotherapy was stopped.

Another Chemotherapy and Palliative Care

Medical Documentation

Vincristine – Dacarbazine-cyclophosphamide was started again.
IE was given on 27.09.2021.

21.10.2021: Vincristine + dactinomycin + cyclophosphamide has been administered.

18.11.2021: Ivo started on Gemzar taxotere treatment for recurrent disease. He was evaluated for course 1 day 8 treatment. HB7.6. 1U of ES can be transfused. Vincristine – Dacarbazine-cyclophosphamide was started again. IE was given on 27.09.2021.

21.10.2021: Vincristine + dactinomycin + cyclophosphamide has been administered. 18.11.2021 Ivo started on Gemzar taxotere treatment for recurrent disease. He was evaluated for course 1 day 8 treatment. HB7.6. 1U of ES can be transfused.

21.10.2021: Vincristine + dactinomycin + cyclophosphamide has been administered.

18.11.2021: Ivo started on Gemzar taxotere treatment for recurrent disease. He was evaluated for course 1 day 8 treatment. HB7.6. 1U of ES can be transfused.