Here, we report the case of a 62-year-old man with neuroblastoma, which is extremely rare in adults. His tumor was resected, but it recurred four months later. Radiotherapy reduced tumor size, and the patient remained in good health three years after surgical tumor removal. The residual tumor and the treatments administered to this patient were evaluated. We have also reviewed the literature.
Neuroblastoma is one of the most common tumors in children, but it is incredibly rare in adults. The disease in adults differs from that in children with respect to tumor markers, treatment, and prognosis. An adult case of neuroblastoma generally has a poor prognosis, and no standard therapy has been established. In general, adult neuroblastoma is only minimally responsive to current therapeutic schemes.
A left adrenal mass was discovered in a 62-year-old male patient during evaluation for mild epigastralgia that had lasted for several days. He also had a 1-year history of mild hypertension, which was not treated. The patient denied having headaches, palpitations, or excessive/inappropriate perspiration. His physical examination was unremarkable, and no cushingoid features, petechiae, or abdominal striae were detected. Serum electrolytes, blood urea nitrogen, creatinine, and a complete blood count were normal. Pertinent adrenal serum and urine chemistries were also normal, except for a slight elevation in serum norepinephrine. An abdominal X-ray revealed an irregular mass with calcification just above the left kidney. Computed tomography (CT) confirmed the presence of a 7 × 6 cm mass containing sporadic calcification that was located in the left suprarenal position above the left kidney (Figure
Abdominal computed tomography (CT) image of a left adrenal mass. (a) A preoperative CT scan revealing a 7 × 6 cm tumor with calcification. (b) A CT scan taken 4 months after surgical tumor removal showing a 12 × 9 cm recurrent tumor. (c) Three years after radiotherapy, the residual tumor was 4 × 3 cm.
The patient underwent a left adrenalectomy and a splenectomy that was performed via a thoracoabdominal incision. The mass was partially encapsulated, but the inferior and posterior portions were strongly adherent to adjacent tissues. Gross inspection showed that the tumor measured 8.5 × 5 × 4 cm and 115 g. The cut surface revealed a grayish solid portion and a dark reddish necrotic portion. Histopathologically, the tumor was composed of eosinophilic filamentous tissues and small round cells with hyperchromatic nuclei and scant cytoplasms (Figure
Photomicrographs revealed various tumor properties. (a) The tumor contained small hyperchromatic cells mixed with a finely fibrillar stroma (hematoxylin and eosin). The tumor stains positively for (b) neuron-specific enolase, (c) vimentin, (d) synaptophysin, and (e) S-100 protein. All presented images were reduced from ×400 magnification images.
Four months after surgical removal of the tumor, CT revealed a recurrent abdominal mass that was 12 × 9 cm in size (Figure
Three years after surgery, the patient remains well without any metastases. The mass has decreased to 20% of its original size, with the central necrotic portion of the tumor measuring larger and the peripheral parenchymal portion measuring smaller (Figure
Neuroblastoma is a relatively common solid tumor in children, comprising about 8% of all childhood cancers [
A literature review revealed that there have been 190 adolescent and adult cases of neuroblastoma described from 1987 to 2014 [
Neuroblastoma in adolescents and adults has been reported to lack MYCN oncogene amplification, which occurs in 20–30% of younger children. Elevation of catecholamine metabolite secretions, including urinary vanillylmandelic acid (VMA), homovanillic acid (HVA), and dopamine, occurs in as many as 90% of pediatric patients [
The majority of neuroblastomas are irregularly shaped, lobulated, and unencapsulated on CT and MRI imaging. They also sometimes invade adjacent organs or encase adjacent vessels. Neuroblastomas tend to be nonhomogeneous because of tumor necrosis and hemorrhage. They also contain calcifications in approximately 85% of cases, as determined using CT imaging [
The rarity of neuroblastomas in adolescents has led to the lack of systematic trials for chemotherapeutic agents, either alone or as a part of combination therapy. CYVADIC (cyclophosphamide, vincristine, adriamycin, and dimethyl triazeno imidazole carboxamide) and James (cyclophosphamide and vincristine) regimens have been administered to adults, but these protocols have not been analyzed because of limited data. Castleberry et al. [
Spontaneous maturation and tumor regression have been documented at a much higher rate in patients with neuroblastoma than those with any other type of tumor [
The authors have no conflict of interests to declare regarding the material and methods reported in this paper.