A complex surgery on a nine-day-old newborn was successfully performed at Acibadem Sistina Hospital. During the pregnancy, the baby was diagnosed with a tumor mass on the right adrenal gland, suspected to be neuroblastoma.

Pediatric surgeon Assoc. Prof. Risto Simeonov, MD, PhD, who performed this extremely complex intervention, explains that it is a very rare condition requiring rapid diagnostics, a multidisciplinary approach and ultimate surgical precision.

“These tumors most often develop after birth and are extremely rarely diagnosed while the fetus is still in the womb, and surgeries on babies only a few days old are even rarer. In highly developed European centers, such as the ‘Bambino Gesù’ Pediatric Hospital in Italy and the ‘Great Ormond Street Hospital’ in London, only 5 to 10 such cases undergo surgery annually”, Simeonov, MD, points out.

The surgery was high-risk due to the size and specific location of the tumor. Such interventions require maximum calm, precision and experience by the entire team, especially the surgeon and anesthesiologist.

MULTIDISCIPLINARY APPROACH TO TREATMENT

Neuroblastoma in children requires treatment in highly specialized centers for pediatric surgery and pediatric oncology, involving a multidisciplinary team consisting of pediatric surgeons, pediatric hematologists-oncologists, neonatologists, radiologists, anesthesiologists and pathologists.

Acibadem Sistina Hospital has the necessary resources for the treatment of such complex cases available: modern diagnostics, an experienced medical team, surgical capacities, neonatal and pediatric intensive care units, as well as continuous postoperative monitoring.

During a regular ultrasound examination in the 36^th week of gestation in a 24-year-old patient from Skopje, who regularly followed her pregnancy at another institution, a tumor formation on the adrenal gland in the fetus was detected. The patient sought a second medical opinion at Acibadem Sistina Hospital. She was admitted to the Department of Gynecology and Obstetrics by Milica Kalamaras, MD, a specialist in gynecology and obstetrics, who confirmed the diagnosis and performed the delivery in the 39^th week of gestation.

Even before the baby was born, in coordination with the pediatric surgeon Assoc. Prof. Risto Simeonov, MD, PhD, a multidisciplinary team for further monitoring and treatment was formed. Immediately after birth, the newborn was cared for at the Department of Neonatology under the supervision by Simonida Spasevska, MD, a specialist in pediatrics. All necessary tests were immediately initiated to accurately determine the diagnosis and plan the relevant treatment.

TUMOR THE SIZE OF A BABY'S HEAD REMOVED

The magnetic resonance imaging and biopsy, performed by Prof. Zoran Trajkovski, MD, PhD, a specialist in radiodiagnostics, indicated that the tumor mass had significantly increased after birth. In order to accurately determine a possible spread of the disease in the bone marrow, Assoc. Prof. Biljana Choneska-Jovanova, MD, PhD, pediatric hematologist-oncologist, performed a bone marrow biopsy.

“Prenatally, the tumor had a diameter of about five centimeters. However, the magnetic resonance imaging performed after the birth detected that the mass had more than doubled, reaching a diameter of as much as 11 centimeters”, explains pediatric surgeon Assoc. Prof. Risto Simeonov, MD, PhD.

As per the European medical recommendations, prenatally diagnosed neuroblastomas in principle should be monitored, there is a rule of monitoring the condition and waiting. In this newborn, however, the rapid tumor growth and its impact on the baby's health required urgent surgical intervention.

"In prenatally diagnosed neuroblastomas, there are strictly established rules for monitoring and treatment. But when the tumor increases by more than 40 percent, the possibility of surgical treatment must be considered. In our case, its dimensions had increased by more than 100 percent. The tumor exerted strong pressure on the large blood vessels, vena cava and aorta, causing impaired circulation and difficulty breathing. Therefore, we decided for emergency surgery", explains Assoc. Prof. Simeonov, MD, PhD.

Assoc. Prof. Risto Simeonov, MD, PhD, formed the surgical team in the shortest possible time. Zlatko Aleksovski, MD, a specialist in pediatric surgery, and Milcho Micevski, MD, a specialist in anesthesiology, participated in the intervention as well.

“The patient underwent a right-sided adrenalectomy i.e. complete removal of the tumor along with the right adrenal gland. The surgical material was sent for histopathological analysis, the result of which confirmed the final diagnosis. The greatest challenge in such interventions is to remove the tumor completely, without damaging the surrounding vital organs, the right kidney and liver, without injuring the large blood vessels, vena cava and aorta, since the surgery can be fatal. The operative and postoperative courses went normally, and the patient was discharged in good general and local condition, with recommendations for further care and regular follow-ups”, says Assoc. Prof. Simeonov, MD, PhD.

Following the successfully completed surgery, the baby was transferred to the Neonatal Intensive Care Unit, where intensive monitoring and postoperative treatment continued.

According to Assoc. Prof. Choneska-Jovanova, MD, PhD, the sample obtained by bone marrow puncture biopsy did not determine presence of malignant cells. Additionally, bone marrow and tumor tissue samples were analyzed with fluorescence in situ hybridization (FISH) to determine possible presence of MYCN oncogene amplification.

“No MYCN oncogene amplification was detected in the analyzed samples. Combined with the favorable histology, localized disease, absence of distant metastases, and complete surgical removal of the tumor, it represents an extremely favorable prognostic factor. It is a low-risk neuroblastoma, without necessity for additional chemotherapy or radiotherapy. Probability of a permanent recovery is very high, and regular follow-ups, including imaging procedures and laboratory tests, are necessary in the future”, explains Assoc. Prof. Choneska-Jovanova, MD, PhD.

GRATITUDE TO THE MEDICAL TEAM

Following the successful surgery, the family expressed their sincere gratitude to the doctors, nurses, and all staff for the rapid response, expertise, and support they were provided during their hospitalization.