|Year : 2018 | Volume
| Issue : 1 | Page : 41-43
Sacrococcygeal teratoma with organoid lung differentiation: A rare entity
Mamta Jain, Rajeev Saxena, Sangeeta Saxena, Sanjay Jain
Department of Pathology and Pediatric Surgery, Government Medical College, Kota, Rajasthan, India
|Date of Web Publication||6-Feb-2018|
Dr. Mamta Jain
W/o Dr. Abhishek Jain, C/o Manish Book Depo, Rampura Bazaar, Kota - 324 009, Rajasthan
Source of Support: None, Conflict of Interest: None
Teratoma is a tumor that is derived from three germ cell layers, and it occurs commonly in the ovary and sacrococcygeal region. In the present study, we report a rare case of sacrococcygeal teratoma of newborn showing features of organoid lung differentiation. A 2-day-old girl child presented with left gluteal mass. Surgical excision was done, and histopathology revealed mature sacrococcygeal teratoma with adult type organoid lung differentiation which is a very rare finding.
Keywords: Miniature lung formation, organoid lung differentiation, sacrococcygeal teratoma
|How to cite this article:|
Jain M, Saxena R, Saxena S, Jain S. Sacrococcygeal teratoma with organoid lung differentiation: A rare entity. J Clin Neonatol 2018;7:41-3
|How to cite this URL:|
Jain M, Saxena R, Saxena S, Jain S. Sacrococcygeal teratoma with organoid lung differentiation: A rare entity. J Clin Neonatol [serial online] 2018 [cited 2022 May 19];7:41-3. Available from: https://www.jcnonweb.com/text.asp?2018/7/1/41/224816
| Introduction|| |
Sacrococcygeal teratoma is defined as a tumor emerging from the proliferation of pluripotent cells, containing tissues derived from one or more germinal cell layers.
The incidence of disease is between 1/35,000 and 1/40,000 live births, and female-to-male ratio is 3:1–4:1.
Organoid development of pulmonary tissue is quite rare in teratomas.
Only 8 cases have published. Five of which have been described in the ovary, two in spine, and one in uterine cervix but not in the sacrococcygeal region yet. Therefore, the presence of mature adult type of organoid lung tissue as in the current case is a rare finding.
The present case is to the best of our knowledge is the first case, in which mature teratoma having adult type organoid lung tissue in the sacrococcygeal region.
| Case Report|| |
A 2-day-old girl child presented to pediatric surgical outdoor with a visible lump of approximately 5-cm size at the top of buttock crease following normal delivery. No antenatal records were available; however, no significant antenatal history was there. On physical examination, no other associated malformation or any abnormality was seen.
USG of gluteal swelling revealed a partly solid and partly cystic mass of 6.6 cm × 4.3 cm size extending from abdomen to pelvic area. It is lying anterior to the coccyx and posterior to the rectum.
USG Abdomen, routine hematological and biochemical investigations were within normal limits.
The baby was planned for surgery, and complete excision of tumor was done, and then sent for histopathological examination. Postoperative recovery was uneventful.
Macroscopic examination of specimen showed 3 irregular tissue pieces out of which 2 were cystic and one piece was solid mass 3 cm × 3 cm × 1.5 cm. The cut surface was dark brown [Figure 1].
|Figure 1: Three irregular tissue pieces out of which 2 were cystic. Cut surface is gray brown|
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Microscopy revealed features of benign cystic teratoma showing cysts lined by pseudostratified ciliated columnar epithelium, island of immature cartilage, mature bony tissue, mucus gland, and intestinal epithelium. Well-differentiated lung tissue showing bronchioles and alveoli were seen [Figure 2].
|Figure 2: Hematoxylin and Eosin section reveal cyst lined by pseudostratified columnar epithelium with island of cartilage and mature well-developed alveoli|
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| Discussion|| |
Sacrococcygeal teratomas are the most common solid tumor found in newborn babies (neonates). Tumors located in the sacrococcygeal region have been reported since the 19th century. The first review to elaborate on sacrococcygeal teratoma was by Deveer and Browder in 1937.
Keslar et al. reported that 69 (62%) of 96 sacrococcygeal teratomas in their series were composed of both solid and cystic component.
Mature glial tissue, skin, respiratory, enteric epithelium, cartilage, smooth muscle, and striated muscle are the most common element found. Bone, pancreatic tissue, choroid plexus, and adrenal tissue are less commonly identified.
Organoid development of lung in teratoma is very rare. Only 8 cases have been published. Five of which have been described in the ovary, two in spine and one in uterine cervix but not in the sacrococcygeal region yet. Therefore, the presence of mature adult type of organoid lung tissue as in the current case is a rare finding.
The present case is to the best of our knowledge is the first case, in which mature teratoma having adult type organoid lung tissue is in sacrococcygeal region.
The symptoms depend on the size and specific location of the tumor. Small tumors are often asymptomatic and can usually be removed surgically after birth without difficulty as was evident in this case. It should always be excised soon after delivery to avoid tumor ulceration, hemorrhage, and risk of malignant change.
The cause of sacrococcygeal teratomas is unknown. Various theories have been proposed for the origin of this tumor, also known as extragonadal germ cell tumor. The most accepted is that it arises from an area under the coccyx called “Henson's Node” where germ cells persist which can give rise to cells of the three major tissue layers of an embryo.
Clinically, this tumor can simulate myelomeningocele, chordomas, tumors consisting of fat (lipomas), extrarenal Wilms' tumor, hamartomas, and neuroblastomas. Antenatal ultrasonographyand histopathological evaluations can help in confirming the diagnosis. In the present case, since sonography was not done, it could not be diagnosed. Antenatal diagnosis helps in decreasing the mortality and morbidity of the fetus and also mode of delivery can be planned depending on the size of tumor.
| Conclusion|| |
Here, we are presenting a rare case of sacrococcygeal teratoma showing organoid lung differentiation.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for images and other clinical information to be reported in the journal. The patient's parents understand that the name and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
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[Figure 1], [Figure 2]