Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2020  |  Volume : 25  |  Issue : 2  |  Page : 85--90

Clinical presentation and surgical management of neonatal tumors: Retrospective analysis


Shailesh Solanki1, Prema Menon1, Ram Samujh1, Kirti Gupta2, KL N. Rao1,  
1 Department of Pediatric Surgery, PGIMER, Chandigarh, India
2 Department of Pathology, PGIMER, Chandigarh, India

Correspondence Address:
Dr. Prema Menon
Block 3A, Room No. 3103, Advanced Pediatric Centre, PGIMER, Sector 12, Chandigarh - 160 012
India

Abstract

Aims: Neonatal tumors (NTs) include a group of diverse neoplasms. In this study, we reviewed our data for clinical presentations, management options, and outcome. Materials and Methods: All patients from 0- to 1-month age presenting with solid tumors, from 2006 to 2018 were studied. The gender, presentation, location, type of tumor, and management were analyzed. The final diagnosis was made with histopathology in all cases. Hemangiomas and lymphangiomas were excluded from the study. Results: A total of 32 neonates were studied. The most common tumor was sacrococcygeal teratoma (SCT,16) followed by teratoma at other sites including two cases of fetus-in-fetu, soft-tissue sarcoma (STS, 4), mesenchymal hamartoma (2), hemangioendothelioma (2), and other rare tumors. Three tumors were diagnosed antenatally; of whom, two were neither visible externally nor palpable. Complete surgical excision was done for all except in a case of ovarian cyst where near-total cystectomy was done. No patient received chemotherapy or radiotherapy. Six patients had postoperative complications, including two who had local recurrence requiring excision. There was one mortality. All the other patients are doing well during follow-up. Conclusion: NTs have varied presentations. SCT and STS were the most common benign and malignant tumor, respectively. Early diagnosis and complete surgical excision are often curative for all, regardless of the pathology with the minimal role of chemotherapy or radiotherapy.



How to cite this article:
Solanki S, Menon P, Samujh R, Gupta K, N. Rao K L. Clinical presentation and surgical management of neonatal tumors: Retrospective analysis.J Indian Assoc Pediatr Surg 2020;25:85-90


How to cite this URL:
Solanki S, Menon P, Samujh R, Gupta K, N. Rao K L. Clinical presentation and surgical management of neonatal tumors: Retrospective analysis. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2020 Apr 9 ];25:85-90
Available from: http://www.jiaps.com/text.asp?2020/25/2/85/276942


Full Text



 Introduction



Neonatal tumors (NTs) include a group of diverse tumors, which are diagnosed within the first 30 days of life or antenatally. The incidence of a malignant tumor is 1 in every 12,500–27,500 live births, accounting for 2% of all childhood cancers.[1] The aim of this study is to know the occurrence of the various NTs, their clinical presentations, and management options from our own data.

 Materials and Methods



This was a retrospective study from June 2006 to 2018, conducted at a Pediatric Surgery unit of a tertiary care referral center. Data were collected from previous records maintained by us. Ethical clearance was obtained from the Institutional Review Board (NK/4587/RS/499). We included all patients with solid tumors who presented to our center between 0 and 1 month age including antenatally diagnosed cases. We excluded patients with incomplete data, those lost to follow-up, vascular tumors (hemangioma and lymphangioma) and without tissue diagnosis. Patients who underwent surgery for suspected tumor but not confirmed on subsequent histopathology were also excluded from the study. The final diagnosis was made with histopathology in all cases. The age at the time of surgery, sex distribution, clinical presentation, type of tumor, surgical intervention, complications, and follow-up were assessed.

All children underwent baseline blood investigations and radiological imaging; other investigations were guided by clinical examination findings and suspected pathology. Fine-needle aspiration cytology (FNAC) was done in all cases of soft-tissue tumors (STTs) but not for lesions such as sacrococcygeal teratoma (SCT). Computerized tomography (CT scan) or magnetic resonance imaging (MRI) was advised, depending on tumor location and the involvement of surrounding structures. Our routine protocol for children with SCT was to perform a contrast-enhanced CT (CECT) abdomen and pelvis unless we had a suspicion of intraspinal component, in which case, MRI was preferred. In tumors, involving the face, neck, and extremities, MRI was advised to look for the involvement of local structure, vascular supply, and feasibility of complete excision. Tumor markers (alfa-fetoprotein [AFP], β-human chorionic gonadotrophin, lactic dehydrogenase, and others) were advised as a baseline workup, wherever indicated. We aimed to perform complete surgical excision wherever feasible. Children were kept on long-term follow-up. Tumor markers and radiological investigations were repeated as per indication.

 Results



A total of 32 cases were included in the study. The female-to-male ratio was 1.13:1. The mean age of presentation was 14 days (range 0–28 days), and the mean age at operation was 32 days (range 2–60 days). None of the patients received chemotherapy or radiotherapy either preoperatively or postoperatively. The follow-up ranged from 3 months to 10 years (mean = 3.6 years).

Teratoma

A total of 16 patients were diagnosed with SCT, [Table 1] and [Table 2]. One neonate with giant SCT [Figure 1] had a recurrence, diagnosed by a raised AFP at 5 months follow up. Following a CECT scan, the child underwent excision of a residual mass measuring, 7 cm × 4 cm. She is doing well at 2 years follow-up. Three children with cervicofacial teratoma [Figure 2] presented with obvious mass lesion and tissue diagnosis was made preoperatively by FNAC, underwent complete surgical excision, and the histology was suggestive of mature teratoma with margins free of tumor. All did well during follow-up with no recurrence. One neonate with retroperitoneal teratoma (fetus-in-fetu) diagnosed antenatally, was asymptomatic postnatally and had a nonpalpable tumor. CECT showed a 6 cm × 6 cm solid cystic lesion behind the mesosigmoid, near the aorta, and renal vessels. Another child presented with a mass over the back (fetus-in-fetu) with a limb-like structure attached to it. Both neonates underwent complete tumor excision with no issue during subsequent follow-up.{Table 1}{Table 2}{Figure 1}{Figure 2}

Malignant and other benign tumors

Presentation and management of malignant and other benign tumors are described in [Table 1] and [Table 3], respectively.{Table 3}

 Discussion



NTs represent a varied group of neoplasms that are rare and may be found in any part of the body. The majority of these tumors are solid homogeneous collections of tissue representing several tumor types. They appear to be mostly of sporadic mesenchymal and embryonic origin, carcinomas being virtually absent in the neonatal period.[2] Although they can arise from any part of the body and can be of different histopathology, still some features of NTs make them relevant to study in a single genre. NTs, arising from soft-tissue developmental abnormality are associated with other congenital abnormality presented in various syndromes such as Beckwith–Wiedemann, and Currarino triad. The origin of the NT is from embryonic tissue as a result of intrinsic dysfunction of cellular growth and proliferation.[2],[3] NTs have different behavior patterns, histological features, and treatment responses compared to older children. The features of a tumor that suggest malignancy in older children, such as high nuclear-cytoplasmic ratio, high-mitotic rate, and anaplasia, can be present in benign NTs.[3] There are few studies on neonatal solid tumors which have been published as shown in [Table 4].[4],[5],[6],[7],[8]{Table 4}

SCT is the most common congenital tumor as mentioned in reported literature and accounts for 35%–60% of all teratomas.[9] In our study also SCT was the most common, whereas in some other studies, neuroblastoma was the most common NT.[4],[5],[6] Altman has classified these tumors into four types depending on its external and intrapelvic component.[10] As described in the literature, in our study also, type I (10/16) was the most common then type II (5/16) and one neonate had type III, whereas there was no patient with type IV. SCTs usually have solid and cystic components; although, purely cystic types can occur in approximately 15% cases.[11] In our study, we had two neonates (2/16, i.e., 12.5%) with pure cystic variety and both were benign. These tumors are more prone to rupture during delivery and surgical dissection. To minimize risk of rupture, cesarean delivery is recommended for fetuses with SCTs of more than 5 cm in diameter. In case of large cystic lesion, antenatally, cyst aspiration has been reported with some success.[12] Although most neonates with SCT are asymptomatic, some require intensive care because of prematurity, high-output cardiac failure, disseminated intravascular coagulation, and tumor rupture or internal bleeding.[13]

In this study, we found that large-size tumors (approximately, 20 cm) [Figure 1] had more complications; one child presented with hypovolemic shock due to intratumoral hemorrhage, another neonate developed local recurrence and another neonate developed fecal incontinence in the postoperative period [Table 3]. Another important point for large tumor, especially for teratomas is study of histopathology from maximum possible sites and from all margins. The microscopic foci of yolk sac tumor at any one place can change the prognosis.

In our study, postoperative wound infection/dehiscence was present in three cases, which were managed conservatively. The proximity of anal verge and wound leads to inevitable fecal soiling which was the main reason for wound infection, despite barrier dressing.

Histologically, teratomas are classified as mature or immature on the basis of the presence of immature elements such as neuroepithelial tissue and immature mesenchyme.[14] In our study, three SCTs had immature histology but considering age and complete excision with a negative margin, the pediatric oncology team had decided for close observation and none of the children received chemotherapy. All children had more than 2 years follow-up and are doing well. Similarly, Heerema-McKenney et al. also reported, no need of chemotherapy after complete excision in their study on congenital teratoma. Of 22 teratomas, 15 were survivors with 10 having immature histology. None of the neonates received chemotherapy after complete excision, and there was no recurrence after a follow-up (mean) of 37.6 months.[14]

Cervicofacial teratoma accounts for 2%–5% of all childhood teratomas including a spectrum of lesions that may be life-threatening mainly because of their location. Airway management may be required at birth.[15] In this study, 14% (3/21) of teratomas were in this group [Figure 2]. They are mostly benign, and surgical excision is the treatment of choice. The difficulty for facial teratoma is wound closure after complete excision, wherein cosmesis may be compromised.

Fetus-in-fetu is a rare condition that has been defined as the presence of one of the twins in the body of the other. It is most frequently located in the retroperitoneal area; however, it has been reported in other locations as well.[16] In our study also, one child had teratoma at the back with a well-formed limb-like structure. These are mature teratomas and are mostly benign but with time, malignancy can develop. In our study, one neonate was diagnosed antenatally, and hence before the child became symptomatic or tumor became too large, it was completely excised. Definitely, in this type of antenatally diagnosed cases prognosis becomes better. Complete excision of tumor and histological confirmation of benign tumor is the complete treatment.

STTs, benign and malignant are relatively common and grouped together. They occur particularly in the head, neck, and extremities and are locally aggressive to varying degrees. In our study, all children presented with a rapidly growing mass that distorted the local anatomy [Figure 3]. The surgical challenge is complete excision along with closure of wound and obtains good cosmesis. Histological appearance of congenital fibrosarcoma is no different from that observed in the older child or adult, it seldom metastasizes and has a good prognosis, although radical surgery may be required to achieve local control. Rhabdomyosarcoma, the most common soft-tissue malignancy in older children, is very rare in the neonatal period, although presentation, prognosis, and treatment are similar.[17]{Figure 3}

Mesenchymal hamartoma (MH) is a rare, developmental tumor, with the occasional risk of malignancy; although at times, they have been classified as nonneoplastic developmental anomaly. MH of the liver is the second-most common hepatic tumor in newborns, contain elements of normal hepatic parenchyma including mesenchyme, portal venous branches, bile ducts, hepatocytes, and cysts of varying size.[18] The rapid expansion of the tumor as seen in our case, was likely due to the degeneration of the mesenchyme and fluid accumulation. Surgical resection has been the standard treatment for this tumor.[19],[20]

MH of chest wall originates from the ribs usually and may be multifocal. Chest wall deformity or mass and respiratory distress are the usual presentation as noted in our index case also. Surgery is indicated in the neonatal period itself if there is compression by the mass causing pulmonary or cardiac compromise.[21],[22],[23]

Hemangioendothelioma is a rare vascular tumor, originating from vascular endothelial or pre-endothelial cells. It represents <1% of all vascular tumors. They have a borderline biological behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Complete excision is the treatment of choice as done in the two cases treated by us.[24] There are few case reports mentioning conservative management also.[25]

Congenital mesoblastic nephroma (CMN) is the most frequent type of renal tumor in the neonatal and early infantile period, comprising 3%–10% of all childhood renal tumors. In our case, the mother had noticed the abdominal lump at 3 weeks of age although the child was asymptomatic. CMN has three pathological variants: classic CMN, cellular CMN, and the mixed variant. Cellular CMN as reported in our case, is associated with the potential for malignancy and is capable of recurrence and metastasis.[26],[27] However, surgical resection with nephrectomy is considered adequate therapy for all subtypes, provided complete resection is achieved as in our case.

Ovarian cysts are the most common masses identified in neonatal girls and are nearly always benign. Simple cysts <4–5 cm in size can be observed for spontaneous regression with serial ultrasonography. Preservation of ovarian parenchyma is the hallmark of surgery for ovarian cysts as we did in our case. Options for surgical management include; cyst unroofing, partial or total cystectomy, and oophorectomy. Pathologic evaluation of a portion of the cyst wall is recommended; however, the presence of an underlying malignancy is exceedingly rare.[28],[29] In our case, it was antenatally diagnosed and was increasing in size. Due to its size (9 cm × 7 cm), there was a risk of torsion, and hence neonate underwent ovarian preserving near-total cyst excision at day 6 of life.

The use of chemotherapy and radiotherapy in neonates is associated with a higher incidence of complications and can potentially affect the child's subsequent development. Irradiation is also implicated in the development of second malignancies.[30],[31] In our study, no patient was subjected to chemotherapy or radiotherapy.

There were some limitations in our study. The rarity of NTs limits the analysis by diagnostic groups.[1],[3],[4],[5],[6],[7] Retrospective studies have some of its own limitations and in some patients follow-up was not adequate.

 Conclusion



NTs have varied presentations. SCT and STS were the most common benign and malignant tumor, respectively. Early diagnosis and complete surgical excision are often curative for all, regardless of the pathology, with minimal role of chemotherapy or radiotherapy.

Financial support and sponsorship

Nil.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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