Mature Sacrococcygeal Teratoma Research Articles

Antenatal and histological diagnostics of cystic sacrococcygeal teratoma. Clinical case and literature review

Abstract Objectives The congenital embryonic tumor known as sacrococcygeal teratoma (SCT) affects 1 in 35.000–40.000 newborns and is more prevalent in female fetuses and neonates. A total of 25–50% of SCTs are diagnosed by an ultrasound (US) examination during the second trimester of pregnancy. Planning the manner of delivery, determining the risk of negative outcomes, and choosing treatment options depend on the results of antenatal differential diagnosis. Case presentation This is a unique case of a 29-year-old second gravida, suspected of having a fetal sacrococcygeal dysplasia differentiable between Type 2 SCT and terminal myelocystocele. An MRI revealed no typical SCT changes, as a matter of course, the diagnosis of myelocystocele could not have been excluded. The results of the genetic examination allowed to exclude the chromosomal pathology. Punctuation of the external component of the formation and a cytological examination were suggested. Nevertheless, the patient and her partner refused further studies and insisted on the termination of pregnancy. Medical abortion was induced and histological findings confirmed fetal morphology to be mature SCT. Conclusions Cystic sacrococcygeal teratoma is an unusual malformation of fetal development. In the antenatal period SCT is diagnosed based upon an ultrasound evaluation, an MRI, and a multidisciplinary assessment of clinical experts. Differential diagnosis based upon clinical imaging during the gestational period is elaborate. The final medical diagnosis needs to be verified by a histological evaluation of pathological tissue. An antenatal medical diagnosis of fetal dysplasia is considerable for the further prognosis of fetal and newborn development.

A Rare Case of Oligodendroglioma in Sacrococcygeal Mature Teratoma Diagnosed in Preterm Infant

Sacrococcygeal teratoma is the most common congenital tumor in neonates, and is reported in approximately 1/35,000 to 1/40,000 live births. Oligodendroglioma is a rare central nervous system tumor that is usually found in the cerebral hemisphere of young and middle aged adults. When associated with a teratoma, it is mainly identified in ovarian teratoma in adolescents and adults. We describe a rare case of a preterm infant with oligodendroglioma in a mature sacrococcygeal teratoma. The male neonate was born at a gestational age of 30 weeks with a protruding mass in the sacrococcygeal region. Pelvic magnetic resonance imaging showed a sacrococcygeal teratoma of approximately 11 cm comprising fat components and skeletal structure, that extended from the anterior part of the sacrum to the abdominal cavity. Radical resection was performed at 36 days of age. Macroscopically, the resected intra-abdominal mass had the characteristics of a cystic lesion, and the intrapelvic mass was a predominantly solid mixed cystic-solid lesion. Histologically, this solid lesion in the intrapelvic mass was composed of mature glial tissue, which comprised as a proliferation of monotonous cells with small and round nuclei, surrounded by a perinuclear halo (“fried egg” appearance). Additionally, these cells were immunohistochemically positive for glial fibrillary acidic protein. These findings confirmed the diagnosis of oligodendroglioma in sacrococcygeal mature teratoma. After the treatment, no recurrence was observed during the follow-up period, and no additional intervention was required. However, the patient is undergoing treatment for voiding dysfunction caused by a neurogenic bladder.

Solitary vaginal paraganglioma with mature sacrococcygeal teratoma: a rare case report

BackgroundVaginal paraganglioma are rare, atypical, solitary tumors which originate from the female genital tract. Sacrococcygeal teratoma are also rare neoplasms which derive from one (or more) primordial germ cell layers. Here we report a unique case of vaginal paraganglioma with sacrococcygeal teratoma.Case presentationA 44-year-old female experienced paroxysmal hypertension, palpitations and dizziness for almost six years. Enhanced CT and MRI highlighted two abnormal soft tissue lesions located in the left vaginal wall and coccyx anteriorly, and Iodine-131 metaiodobenzylguanidine (131I-MIBG) demonstrated abnormal radioactive uptake in perineum area. Endocrine tests showed elevated plasma normetanephrine (NMN) and 24 h urine norepinephrine. There was a well-circumscribed soft tissue lesion of approximately 3.5 cm in the left lateral vaginal wall which could be palpated during bimanual examination, together with a 1.5 cm tumor in the posterior wall of the rectum. We completely resected the two lesions in stages with the support of a senior gynecologist and general surgeons. Postoperative histopathological examinations suggested the vaginal paraganglioma and mature sacrococcygeal teratoma. Targeted sanger sequencing for the 36 mostly common paraganglioma-related genes, with a depth of 1000x, revealed no mutations. Post-operatively, plasma NMN and 24 h urine norepinephrine returned to the normal range and her symptoms completely disappeared.ConclusionsWe reported an extremely rare case and the successful treatment of functional vaginal paraganglioma coexisting with adult sacrococcygeal teratoma.

Squamous cell carcinoma in a pre-existing adult mature Sacro-Coccygeal teratoma: A rare case with review of literature

We report a case of sacrococcygeal teratoma with squamous cell carcinomatous transformation in a 40-year-old lady. This is an infrequent location for teratoma in adults and malignant transformation has rarely been described. The uniqueness of the present case was the presence of congenital sacrococcygeal teratoma presenting in adulthood with malignant transformation. Keywords: Malignant transformation, Sacrococcygeal, Squamous cell carcinoma, Teratoma.

A rare presentation of Sacrococcygeal Teratoma as recurrent natal cleft abscess in childhood

We report the case of a seven year old girl presenting with recurrent left natal cleft abscess who was diagnosed with Sacrococcygeal Teratoma (SCT) after having multiple Incision and Drainages (I&Ds). Diagnosis was made with intraoperative digital rectal examination (DRE) and MRI Pelvis. Complete surgical resection was performed and histopathology identified mature SCT with clear margins throughout the entire specimen. At nine month follow-up, she remained well with no evidence of recurrence. To the author's knowledge, this is the first case report of SCT presenting as recurrent natal cleft abscess in a child.

Malignant recurrence after mature Sacrococcygeal teratoma: A meta-analysis and review of the literature.

Sacrococcygeal teratoma (SCT) is a rare extragonadal germ cell tumour mostly diagnosed during infancy and early childhood. Neonatal SCTs are mostly mature, but can also contain immature and/or malignant components. Recurrence of an SCT alters prognosis, especially when it is malignant, of which its mechanism is not yet fully understood. This study is a review and meta-analysis of the literature on malignant recurrences after an initially mature SCT. A literature search was performed to identify studies describing children with SCT and presenting specific information on histology of the initial tumour as well as the recurrence. Random effect models for mature recurrence and malignant recurrence after an initially mature SCT were employed to pool study-specific percentages in order to estimate an overall percentage and its associated 95 % confidence intervals (CI). Inverse variance method, which gives more weight to larger studies, was used to pool outcomes for the different studies. A total of 22 articles, comprising 1516 patients with SCT, were included in the meta-analysis. The pooled proportions of mature and malignant recurrences after mature SCT were 3 % (95 % CI 1-4 %) and 5% (95 % CI 3-6 %), respectively. Fifty-seven (56 %) of a total of 102 recurrences after resection of an initially mature SCT were malignant, mostly yolk sac tumour (YST). Many recurrences occurred within 1-6 years, however some occurred as long as 20 years after initial diagnosis. A substantial number of recurrences of mature SCT present as a malignant tumour. Overlooking malignant components on initial pathological evaluation and the progression of mature SCT cells to malignant cells may play a role. Treatment of mature SCTs with resection alone requires thorough follow-up of at least 6 years. Future research is needed to determine whether SCTs with malignant microfoci should be treated or followed-up differently from mature or immature SCTs. In addition, the value of serum biomarkers in follow-up after SCT needs to be further evaluated.

Adult sacrococcygeal teratoma: a retrospective study over eight years at a single institution.

To determine the clinical, imaging, and histological features, and surgical resection modalities and outcomes of adult sacrococcygeal teratoma (SCT). Adult patients with histopathologically diagnosed SCT were enrolled in our hospital between August 2010 and August 2018. Each patient's characteristics and clinical information were reviewed. There were 8 patients in the study (2 males, 6 females) with a median age of 34 years (range, 18-67 years). The time to clinical symptoms was 14 d to 35 years, with a median time of 4 years. Six patients presented with symptoms of sacrococcygeal pain, and four with signs of sacrococcygeal mass and ulceration in the sacrococcygeal region. Six patients were evaluated using a combination of computed tomography (CT) and magnetic resonance imaging (MRI). All patients showed a presacral tumor with heterogeneous intensity on CT images. All patients underwent surgical treatment, including 6 parasacral, 1 transabdominal, and 1 combined anterior-posterior surgery cases. Seven patients were histopathologically diagnosed with benign mature SCT, and have shown no recurrence. One patient had malignant SCT, with recurrence at 84 months after surgery. After a second surgery, the patient had no recurrence within 6 months follow-up after re-resection. Our retrospective study demonstrated: (1) adult SCT is difficult to diagnose because of a lack of typical clinical symptoms and signs; (2) a combination of CT and MRI examination is beneficial for preoperative diagnosis; (3) the choice of surgical approach and surgical resection modality depends on the size, location, and components of the tumor, which can be defined from preoperative CT and MRI evaluation; (4) most adult SCTs are benign; the surgical outcome for the malignant SCT patient was good after complete resection. Even for the patient with recurrent malignant SCT, the surgical outcome was good after re-resection.

Half-life of alpha-fetoprotein in neonatal sacrococcygeal teratoma

BackgroundAlpha-fetoprotein (AFP) is useful as a tumor marker for sacrococcygeal teratoma (SCT). We investigated the half-life of AFP in SCT. MethodsNeonates who underwent surgical treatment for SCT between 1997 and 2016 were included in the study, whereas patients who died before or after surgery or had malignant germ cell tumors were excluded. ResultsFifty-five non-recurrent SCT patients (M:F = 18:37) were enrolled. They underwent surgery on average 7.4 ± 4.1 days after birth. Serum AFP was measured an average 4.25 ± 2.07 times per patient. We obtained 165 half-lives following the formula (M = Mo * (1/2) Δt/T). A positive correlation was observed between half-life and patient age using the formula T1/2 = 0.0597 × days +6.1643 (p < 0.001). It was different from recurrent SCT (T1/2 = 0.1196 × days −0.0633) (p < 0.05). Half-life was different between mature SCT (T1/2 = 0.0671 × days +4.3912) and immature SCT (T1/2 = 0.0433 × days +8.9339) (p < 0.05). ConclusionThe half-life of AFP in neonatal patients with SCT was prolonged in proportion to the age, and it was getting longer in recurrent tumor than non-recurrent tumor. The half-life of AFP was longer in immature teratoma than in mature teratoma. Level of EvidenceIV.

Sacrococcygeal teratoma with organoid lung differentiation: A rare entity

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.

Giant Mature Sacrococcygeal Teratoma in an Adult - a Case Report

Teratoma is a congenital germ cell tumor, composed of a wide diversity of tissue, containing all three germ cell layers. The sacrococcygeal area is the commonest site of extragonadal teratomas in infants, but presents as a rare diagnostic dilemma in adults. We present a rare case of giant mature sacrococcygeal teratoma in an adult female patient.

Low-grade mucinous epithelial neoplasm (intestinal type) arising in a mature sacrococcygeal teratoma with late recurrence as pseudomyxoma peritonei

Mucinous epithelial neoplasms associated with mature teratomas are well described in the gonads, and some examples have presented with clinical pseudomyxoma peritonei. The association between pseudomyxoma peritonei and an extragonadal teratoma is limited to a single case report. We describe a mature teratoma in the sacrococcygeal region associated with an intestinal-type mucinous epithelial neoplasm that recurred with a disseminated intraperitoneal low-grade mucinous epithelial neoplasm and mucinous ascites. To our knowledge, this is the first reported case of a mature teratoma-associated mucinous epithelial neoplasm from any anatomical site with documented late recurrence as pseudomyxoma peritonei.

Epidural metastases from endodermal sinus tumor arising from benign sacral teratoma

The recurrence of benign sacral teratomas is a small but significant possibility. Recurrence as an endodermal sinus tumor (EST) with epidural metastases, however, has not been previously reported. The authors describe a case of a mature sacrococcygeal teratoma in a 4-day-old female patient that recurred after 22 months as an EST with epidural metastases. The child presented with abdominal pain, urinary retention, and difficulty walking. On imaging, a large pelvic mass and an epidural spinal mass were revealed. The patient's alpha-fetal protein (AFP) level was 68,000 ng/ml. Her neurological examination was significant for 3/5 plantar and dorsiflexion strength bilaterally. The patient underwent L-3, L-4, and L-5 bilateral laminectomies followed by subtotal resection of the recurrent pelvic tumor. Pathological testing of samples of both the recurrent pelvic and the extradural spinal tumors led to a diagnosis of EST. The patient underwent four cycles of chemotherapy with normalization of her AFP level to 13 ng/ml. In the weeks that followed, her AFP level steadily rose again to 167 ng/ml. Follow-up imaging revealed no tumor recurrence. The patient underwent a second course of chemotherapy followed by two tandem courses of high-dose chemotherapy with autologous stem-cell rescue. Since completing this therapy the patient has been clinically stable with an AFP level of 1.3 ng/ml for 14 months. At the 1-year follow-up examination, her plantar and dorsiflexion strength had markedly improved to 4+/5 bilaterally. To the authors' knowledge, this is the first case of a mature sacrococcygeal tumor that recurred as an EST and caused spinal canal compromise.

Sacrococcycygeal Teratoma in Adult

Sacrococcygeal teratomas are neoplasias, which are most frequently found in infants, with the sacrococcígea area being the most frequently found extragonadal location. To describe a sacrococcygeal teratoma in a 51-year-old adult patient and discuss the diagnosis and management. The case of a 51-year-old woman who had a sacrococcygeal teratoma. Computed tomography showed a mass containing calcification. Early signs were when she experienced acute lower back pain upon leaning over or sitting. The tumor appeared first as a sacral swelling. The patient also claimed disturbances in her bowel functioning. Computed tomography (CT) showed the tumor and its relationship inside the pelvic cavity. Surgical ablation was performed on the entire tumor and the coccyx was preserved. The surgical block was sent to pathological exam, which showed it to be a mature sacrococcygeal teratoma. A good clinical evolution took place after resection.

Mature sacrococcygeal teratoma: A case report and literature review

Sacrococcygeal teratomas are derived from embryonic germ cell layers. They present mostly in infancy and are extremely rare in adults; with an associated risk of malignancy. Modern imaging technique may be helpful to delineate the extent of the mass but surgical excision is generally indicated at the time of detection. A case report is presented with a review of literature utilising MEDLINE, Microsoft Net and Yahoo search engines. A three day old female baby presented with a mature sacrococcygeal teratoma containing well-developed limb buds. She had surgical excision and primary repair with good results. A two-year follow up utilising serial serum alpha-fetoprotein assay and CT Scan revealed no evidence of tumour recurrence. Sacrococcygeal teratoma is a rare tumour that may be benign or malignant. Complete excision is the primary therapy and is adequate if the tumour is benign. Chemotherapy and radiotherapy are however indicated in malignant cases and in recurrence after previous excision.

Dystocia caused by Sacrococcygeal Teratoma: A Case Report

A 23-year-old pregnant mother presented with labor pain for 14 hours with delivery of part of a baby with the exception of right leg. Single ultrasonography (USG) done at 34 weeks of gestation showed an anechoic area measuring 8 cm x 6 cm surrounded by a thin rim. The patient was put under general anesthesia (GA) and the right leg of the baby was drawn out of the uterus. A soft tissue mass partly cystic in nature, connected with the perineal region of the baby, prevented the complete delivery. After decompressing the mass, complete delivery was possible. But exploration of the uterus showed rupture in the left lateral wall. Laparotomy with repair of the uterus was done. Excision of the soft tissue mass of the baby was done one day after delivery and histology of the mass showed mature sacrococcygeal teratoma. Postoperative recovery of both the mother and the baby was good. DOI:10.3329/jbcps.v27i3.4296 J Bangladesh Coll Phys Surg 2009; 27: 169-172