Growing teratoma syndrome: diagnostic challenges and outcomes.

PURPOSE: The aim of this case report is to emphasize the significance of the growing teratoma syndrome. Growing teratoma syndrome is frequently misdiagnosed due to its low prevalence, with an estimated incidence of 19% among all immature ovarian teratomas and a lack of experience among healthcare professionals. It is characterized by the growth of benign tumoral tissue during or after chemotherapy for malignant germ cell tumors. CASE REPORT: Our case is about a 46-year-old patient diagnosed with an immature teratoma who was treated unsuccessfully with surgery and chemotherapy. The patient was then referred to our hospital for a second opinion, where this unknown entity was diagnosed and underwent complete surgical debulking, including abdominal wall resection and subsequent repair. CONCLUSION: Physicians need to be aware of rapidly growing masses during or after chemotherapy because early recognition of this syndrome is essential for the adequate treatment of our patients.

A Rare Case of Intracranial Growing Teratoma Syndrome in a Young Adult.

Intracranial growing teratoma syndrome (iGTS) is a rare phenomenon in patients with non-germinomatous germ cell tumor (NGGCT) after chemotherapy or radiotherapy. It manifests as paradoxical growth of teratomatous components, with multiple cystic lesions on cranial imaging despite normalized tumor markers. This paper presents a 22-year-old male with iGTS, diagnosed one month after chemotherapy against NGGCT. Initially diagnosed with presumptive pineal NGGCT causing obstructive hydrocephalus, the patient underwent endoscopic third ventriculostomy and extraventricular drainage with tumor biopsy followed by two chemotherapy cycles. Despite normalization of tumor markers, follow-up MRI showed increased tumor size with honeycomb-like cystic patterns. The patient underwent suboccipital craniotomy for tumor removal via combined telovelar and infratentorial supracerebellar approaches. The final pathology confirmed mature teratoma. However, postoperative bleeding and left thalamic infarction occurred, resulting in severe neurological deficits. Despite challenges, the patient eventually regained the ability to follow simple commands. To understand iGTS pathophysiology, several hypotheses, including the differentiation of immature components and the uninhibited growth of mature components induced by chemotherapy or radiotherapy, were explored. Surgical intervention remains as an ideal treatment, while clinical trials investigate chemotherapy options. Frequent imaging follow-ups are crucial for early detection in iGTS for NGGCT patients.

Mature Cystic Teratoma in a Newborn With Down Syndrome.

Retroperitoneal teratomas are uncommon neoplasms that cause diagnostic dilemmas in neonates during the evaluation of abdominal cystic mass lesions. Down syndrome (DS) is a chromosomal abnormality with an extra third copy of chromosome 21. Due to chromosomal instability, DS is inferred to be a cancer predisposition syndrome. The malignancy pattern in DS is unique with higher incidence of hematological malignancies and solid tumors are rarely reported. Down syndrome neonate was incidentally diagnosed with a retroperitoneal cystic lesion along the left kidney during evaluation for poor feeding on ultrasonography, raising suspicion of an adrenal hemorrhagic cyst. CECT abdomen and pelvis revealed complex cystic lesions along the left renal hilum, with the possibility of cystic neuroblastoma, retroperitoneal cystic teratoma, and cystic lymphangioma. Tumor markers were within normal limits. Surgical exploration revealed a left renal hilar solitary cystic mass lesion, which was excised in toto with a probable intraoperative diagnosis of cystic lymphangioma. The postoperative course was uneventful. Histopathological examination confirmed that the lesion was a mature cystic teratoma. The child is doing well postoperatively at the one-year follow-up. Neonatal retroperitoneal teratomas are unusual neoplasms. A favorable outcome can be achieved, with early diagnosis and treatment.

The prenatal imaging of a rare congenital intracranial teratoma.

Fetal intracranial teratoma presents a rare and devastating diagnosis. Typically, this condition is first detected during routine prenatal ultrasounds, appearing as an irregular heterogeneous lesion. Further insights are gained through fetal magnetic resonance imaging (MRI), better characterizing the anomaly. The combination of these modalities provides detail-oriented high resolution MRI images, while follow-up ultrasounds capture dynamic growth changes, serving as a cost-effective and easily accessible adjunct. This fast-growing tumor leads to macrocephaly and ventriculomegaly, causing severe distortion of the brain parenchyma. Early detection is crucial for effective fetal management and preventing maternal complications. Unfortunately, treatment options are limited due to the tumor's aggressive nature, typically resulting in fetal demise shortly after birth. Here, we present the sonographic and MRI findings of a congenital intracranial teratoma, reaching massive proportions and replacing the entire cerebral hemisphere.

Mature congenital intraventricular intracranial teratoma: A case report and literature review.

Background: Intracranial teratomas represent a rare subset of neoplasms characterized by tissues derived from multiple germ layers within the cranial cavity. These tumors, originating from primordial germ cells, exhibit diverse clinical presentations and histopathological features. While predominantly located along the midline axis, including the suprasellar cistern and pineal region, they can also manifest in less common areas such as ventricles and hypothalamic regions. Histopathologically, they are classified as mature, immature, or malignant based on the degree of tissue differentiation. Case Description: Male patient with prenatal care for congenital hydrocephalus born at 38 weeks gestation with a bulging fontanelle. Postnatal imaging revealed an intraventricular lesion, later diagnosed through magnetic resonance imaging as a mature teratoma invading the lateral ventricle and extending to the hypothalamus. Surgical resection achieved total macroscopic removal followed by successful postoperative ventriculoperitoneal shunting due to evolving hydrocephalus. Conclusion: Teratomas are uncommon tumors, and prognosis depends on tumor size and location, especially considering the rarity of mature teratomas. Complete surgical resection is paramount for treatment, leading to a better prognosis and quicker recovery. In cases where complete removal is challenging, adjuvant therapies and cerebrospinal fluid diversion may be required to enhance therapeutic outcomes and ensure successful resection.

Monodermal teratoma: Three case reports and review of literature.

BACKGROUND: The incidence of monodermal teratomas of the reproductive system is low, and most doctors lack adequate understanding, which can easily lead to missed diagnoses and/or misdiagnosis. Therefore, it is important to fully understand the clinical characteristics, diagnosis, differential diagnosis, and treatment of monodermal teratomas of the reproductive system. CASE SUMMARY: Case 1: A 14-year-old boy was admitted to the hospital with a right testicular mass for 1 wk and underwent surgical resection. He was finally diagnosed with right testicular monodermal teratoma with no special postoperative discomfort. Case 2: A 40-year-old woman was admitted to the hospital for uterine abnormalities indicated by ultrasound 20 d prior and underwent laparoscopic surgery. She was finally diagnosed with a left ovarian monodermal teratoma with a satisfactory postoperative quality of life. Case 3: A 49-year-old woman was admitted to the hospital with a pelvic mass that was discovered on B-ultrasound a week prior and underwent laparoscopic resection of the left adnexa. She was finally diagnosed with left ovarian monodermal teratoma, and her postoperative quality of life was satisfactory. CONCLUSION: Monodermal teratoma is a rare tumor whose clinical manifestations are primarily benign. Simple surgical resection of the tumor is effective.

Prostate Type Malignancies Arising in Ovarian Teratomas - A Report of Two Patients.

The identification of benign prostatic tissue within ovarian and testicular mature teratomas is an unusual occurrence. While a few documented reports exist in the literature regarding the emergence of benign prostatic tissue within teratomas, the occurrence of prostatic-type adenocarcinoma in a mature ovarian teratoma is an exceptionally rare phenomenon. To date, only two prior reports have documented such instances, and no tumors have been previously reported with prostate-type tissue with morphologically two different malignancies. We outline our experience with two tumors involving prostatic-type carcinoma, both arising in ovarian mature teratomas. Microscopic examination of the first tumor revealed small areas of infiltrative atypical glandular proliferation within the mature teratoma. In the second tumor, prostate-type tissue exhibited a low-grade basal cell carcinoma. Additionally, adjacent minute foci of adenocarcinoma of the prostate (Gleason score 3 + 4 = 7, <5% pattern 4) were identified. Goblet cell adenocarcinoma of appendiceal type was also evident in the latter tumor. In both tumors, immunostains (NKX3.1, PSA) were performed to establish the prostatic origin of these atypical glands and PIN4 was performed to document the absence of basal cell in the atypical glands. On clinical follow-up, both patients have no signs of recurrence at 14 and 11 months after the surgery. Further reports on such neoplasms would contribute to a better understanding of the prognosis and management of such occurrences.

Immature ovarian teratoma with gliomatosis peritonei, paraneoplastic hyponatremia and growing teratoma syndrome: a case report and literature review.

Introduction and relevance: Paraneoplastic hyponatremia is often secondary to syndrome of inappropriate antidiuretic hormone secretion (SIADH) by tumour cells. Immature ovarian teratomas (IOT) are uncommon and may present with SIADH. Case report: A 26-year-old female presented with a 3-month history of abdominal pain and constipation. Imaging identified a mixed solid-cystic right ovarian mass containing fat and peritoneal deposits. Biochemistry showed severe, refractory hyponatremia (117 mmol/l). She underwent diagnostic fertility-preserving right salpingo-oophorectomy and resection of peritoneal nodules with the aim to achieve symptom control and hyponatraemia resolution. Pathology revealed a FIGO Stage 2 Grade 2 IOT with extensive benign peritoneal gliomatosis. Initial management was conservative. After 6 months of active follow-up, a rise in AFP, and recurrent hyponatremia supported the decision to administer three cycles of Bleomycin-Etoposide-Cisplatin chemotherapy. One month later, given radiological disease progression despite satisfactory biomarker response, cytoreductive surgery with complete macroscopic resection was performed. Pathology consisted solely of peritoneal mature glial elements: a growing teratoma syndrome (GTS). The patient remains disease-free after 2 years of surveillance. Clinical discussion: Specimen histological assessment from the patient's initial surgery showed immature neuroectodermal tubules, which are thought to be the source of vasopressin secretion. The authors hypothesise that recurrent hyponatremia and rising AFP levels represented postoperative disease relapse. Biochemical response despite radiological disease progression was pathognomonic of a GTS. Conclusion: Paraneoplastic SIADH secondary to an IOT must be considered in female patients presenting with abdominal symptoms and hyponatremia. Management requires a multidisciplinary approach. Serum electrolytes are useful surveillance biomarkers supplementary to tumour markers.

Neuroendocrine Lesions Arising From Mediastinal Teratoma-A Case Report and Literature Review.

Background. Neuroendocrine lesions arising from mediastinal teratomas are rare tumors with only small number of patients reported in literature. The behavior of these lesions appears to be different from traditional neuroendocrine neoplasms. A comprehensive review will be valuable for histologic assessment and treatment planning for similar cases. Case presentation. We present an example of a 57-year-old man who presented with cough. Subsequent work-up revealed an anterior mediastinal mass of 2.1 cm on computed tomography. The patient underwent robot-assisted thoracoscopic thymectomy. Histological examination revealed a mature cystic teratoma with a neuroendocrine component consisting of clusters of tumor cells with round to oval nuclei and a "salt-and-pepper" chromatin pattern. The tumor cells were immunoreactive to cytokeratin, synaptophysin, chromogranin, and INSM1, with a Ki-67 proliferative index of 4%. A histological diagnosis was mature teratoma with well-differentiated low-grade neuroendocrine tumor (carcinoid) was made. The patient was well and without disease after complete surgical excision at 10 months. Literature review. Literature reviewed yielded 13 examples of neuroendocrine lesions arising from mediastinal teratomas. No disease-related mortality was reported, even in lesions with high-grade neuroendocrine, carcinomatous, or immature teratomatous components. Conclusions. Surgical removal is the mainstay of treatment of these lesions, and the presence of a neuroendocrine component does not appear to negatively affect prognosis.

Impact of microscopic deposits of yolk sac tumor on recurrence of mature sacrococcygeal teratoma.

INTRODUCTION: Sacrococcygeal teratomas (SCT) with malignant histology frequently recur and are treated aggressively, but risk factors and surveillance protocols are less established for mature tumors. In particular, prior studies have not investigated whether microscopic deposits of yolk sac tumor (YST) in otherwise mature teratomas lead to higher recurrence rates. METHODS: We reviewed patients with mature SCTs resected at our institution from 2011 to 2021 and analyzed tumor characteristics, treatment, and outcomes. RESULTS: We identified 56 patients with mature SCT, of which 9 (16%) demonstrated microscopic YST. Following surgery, 7/56 (13%) patients developed local recurrence at a mean of 1.2 ± 0.7 years, while no patients developed metastases. Recurrence was more likely in patients with microscopic YST [5/9 (56%) vs. 2/47 (4%), p = 0.021] and positive margins [6/24 (35%) vs. 1/32 (3.1%), p = 0.030]. A solid tumor component tended to increase recurrence risk as well [6/29 (21%) vs. 1/27 (4%), p = 0.053]. Five patients demonstrated malignant recurrence and were all detected by a rising alpha-fetoprotein (AFP), while two patients demonstrated recurrence of mature teratoma and were detected on surveillance magnetic resonance imaging (MRI). CONCLUSIONS: Microscopic foci of YST may increase recurrence risk for patients with mature SCT. Such patients might benefit from closer postoperative surveillance with serial AFP measurements and MRI.

Mucinous intestinal adenocarcinoma arising from mature cystic teratoma of the ovary treated with FOLFOX-6 - a case report.

The malignant transformation of mature cystic teratomas during pregnancy is a rare occurrence in medical literature. In this case report, we present a remarkable instance of mucinous intestinal adenocarcinoma arising from a mature ovarian cystic teratoma diagnosed during pregnancy. This is among the few reports detailing the effective use of the FOLFOX 6 chemotherapy regimen for treating this type of intestinal cancer. Furthermore, we emphasize the immunohistochemical results that confirm the colorectal histological origin.

Choroid Plexus Papilloma Tumor of the Ovary: A Case Report.

This is a case report of a 19-year-old nulligravid patient with a choroid plexus papilloma tumor in a mature cystic teratoma in the right adnexal area. The patient, who had abdominal pain and dyspepsia, showed a 9 cm diameter mass with a solid/cystic component, initially interpreted as a dermoid cyst in the right adnexal region. Mature cystic teratoma is a benign germ cell tumor and is common in women during the reproductive period. However, choroid plexus papilloma is a rare brain tumor. The diagnosis of ovarian choroid plexus papilloma can be made with imaging tests such as magnetic resonance imaging or computed tomography, and treatment is usually by surgical removal. Only four cases of ovarian teratoma with choroid plexus papilloma have been informed in the English literature, and this issue is the fifth.

A case report of hepatic fetus-in-fetu: Approach to diagnosis.

Multiple theories have been proposed about the pathophysiology of Fetus-in-fetu (FIF). The most widely accepted theory is abnormal embryogenesis in diamniotic monochorionic pregnancies, in which a malformed parasitic fetus is found within the body of a twin host. Hepatic FIF has been reported in almost 1% of FIF cases, with only 2 case reports being published in the literature. This article presents the third case report of intrahepatic FIF. Additionally, we review the role of radiology in diagnosing these cases and guiding their proper management. This case report supports the monozygotic twin theory of FIF and the diagnostic dilemma of FIF vs. teratoma can be solved through collaborative work between radiologists and pathologists.

Teratoma to Angiosarcoma: A Metamorphosis in the Mediastinum.

We describe a rare and remarkable transformation of an immature mediastinal teratoma into high-grade angiosarcoma in a 21-year-old male. Mediastinal teratomas, particularly immature ones, are exceedingly rare, representing a small fraction of germ cell tumors (GCTs). Our case describes the clinical journey of the patient, who initially presented with acute chest pain and was subsequently diagnosed with an immature teratoma following imaging studies and elevated tumor markers. Despite an initial positive response to cisplatin-based chemotherapy, surveillance imaging revealed liver masses, which a biopsy confirmed as angiosarcoma. This transformation underscores the aggressive nature of immature teratomas and the propensity for sarcomatous differentiation, particularly in the mediastinum. The case contributes valuable insight into the management and surveillance of mediastinal non-seminoma germ cell tumors (MNGCT), a subset of GCTs with limited literature. We believe this case is the first in the literature to describe a transformation from an immature teratoma in the mediastinum to a high-grade angiosarcoma.

Coexisting Sacrococcygeal Teratoma With Mild Encephalitis/Encephalopathy With a Reversible Splenial Lesion: A Case Report.

Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a rare disease characterized by a reversible lesion in the splenium of the corpus callosum (SCC) observed on MRI. The exact etiology of MERS is unknown, although infections and antiepileptic drugs have been reported as potential causes. Herein, we present the case of a 56-year-old male patient who experienced fever and headache for 3 days. He was referred to our hospital after symptomatic treatment by his primary care physician failed to improve his symptoms. The patient had no psychiatric symptoms or significant neurological findings. Head MRI revealed a high signal on SCC on diffusion-weighted imaging, raising the suspicion of MERS. All examinations to determine the cause of MERS were negative. The patient's symptoms improved with antibiotics and B complex vitamins. Upon admission, abdominal CT incidentally revealed a well-defined mass on the dorsal surface of the rectum suspected to be a tailgut cyst, warranting surgical resection. The cranial margin of the tumor was caudal to the third sacrum, and a trans-sacral approach was used for resection. The fifth sacrum and the coccyx were resected, and the tumor was resected without damaging the rectum. A histopathological examination revealed a mature teratoma without any malignancy. A follow-up CT at four months postoperatively showed no evidence of clinical recurrence of MERS. Adult-onset MERS is relatively rare, and no association with tumors has been reported. The association between encephalitis and teratomas includes ovarian teratomas, which cause anti-N-methyl-D-aspartate receptor encephalitis and paraneoplastic limbic encephalitis. Although the cause of MERS was unknown in this case, we report the coexistence of a sacral teratoma and MERS to contribute to the knowledge of the association between them.

The origin of ovarian Leydig cells: a possibly solved enigma?

Over the years, the origin of ovarian Leydig cells has been, and still is, a topic subject to deep debate. Seven years ago, we proposed that this origin resided in intraneural elements that came from a possible reservoir of neural crest cells, a reservoir that may be located in the ganglia of the celiac plexus. We believe we have found the evidence necessary to prove this hypothesis.

Management strategy for children with ovarian immature teratoma: results from a tertiary pediatric oncology center.

OBJECTIVES: We present an Egyptian study on pediatric ovarian immature teratomas (ITs), aiming to clarify our treatment strategy selection. METHODS: A retrospective review of all children with pure ovarian ITs who were treated at our institution between 2008 and 2023. The analysis included clinical characteristics, tumor staging according to Children's Oncology Group (COG), grading based on the Norris system, management, and outcomes. RESULTS: Thirty-two patients were included, with a median age of 9 years. All patients underwent primary surgery. Unilateral salpingo-oophorectomy was performed in 31 patients. Surgical staging was completed in all patients. Based on COG staging, there were 28 patients (87.5%) stage I, 1 (3%) stage II, and 3 (9.5%) stage III. According to Norris classification, 16 patients (50%) were classified as grade I, 9 (28%) grade II, and 7 (22%) grade III. All patients in stage I were treated using surgery-alone approach, whereas the remaining four (12.5%) received adjuvant chemotherapy. Five patients in stage I had gliomatosis peritonei (GP), and none of them underwent extensive surgery. At a median follow-up of 86 months, two patients had events. The first patient (stage III/grade I) developed IT relapse on the operative bed, and the second (stage I/grade I) had a metachronous IT on the contralateral ovary. Both patients were successfully managed with surgery followed by second-line chemotherapy. Five-year overall survival and event-free survival for all patients were 100% and 93.4%, respectively. CONCLUSIONS: Surgery-alone strategy with close follow-up achieves excellent outcomes for localized ovarian ITs in children, irrespective of the Norris grading or the presence of GP. However, adjuvant chemotherapy is questionable for patients with incompletely resected or locally advanced tumors, and its role requires further evaluation through prospective multicentric studies with a larger sample size.

Prenatal diagnosis in estimating the prognosis of sacrococcygeal teratoma.

: Sacrococcygeal teratoma is a rare congenital malformation, the prognosis depends on factors affecting foetal development. The diagnosis is based on ultrasound examination, especially the evaluation of the detailed morphology of the foetus in the 20th week of pregnancy. Therefore, it is crucial to keep looking for ultrasound markers that would prenatally determine the most accurate prognosis for the foetus. Now, we rely on a small number of studies with a predominance of case reports. We offer a literature review of the essential information concerning sacrococcygeal teratoma diagnostics, therapy, and complications of sacrococcygeal teratomas in connection with prenatal diagnosis. It turns out that in cases with a favourable prognosis according to prenatal ultrasound examination and adequate surgical treatment after childbirth, the prognosis of this congenital malformation is excellent.

A rare case of benign retrorectal teratoma in an adult female: Diagnostic challenges and successful surgical management.

INTRODUCTION: Retrorectal tumors are rare growths with various types which are found in the space behind the rectum. They can be diverse and are often diagnosed through imaging and surgery. PRESENTATION: A 31-year-old female patient came to the clinic with concerns about irregular periods and constipation, but no history of abdominal pain, pelvic pressure, or weight loss. She had a previous surgery to remove an ovarian teratoma when she was three months old. Physical exams and lab tests showed no significant findings, except for a pelvic ultrasound that revealed a normal right ovary with a small follicle and a missing left ovary due to prior surgery for a dermoid cyst. Another cyst, measuring 8.2 × 9.3 × 5.7 cm, was found behind the uterus, believed to be a presacral cyst possibly originating from elsewhere. Further investigation with a CT scan confirmed the presence of a large cyst near the rectum, leading to an open surgical procedure to remove it. The cyst, located deep behind the rectum and next to the levator ani muscle, contained a substance resembling cheese with hair, suggesting a benign dermoid cyst with granulation tissue. The surgery was successful, and the diagnosis was confirmed through histopathological analysis. DISCUSSION: Retrorectal teratomas are rare germ cell tumors that mainly affect children, often presenting with vague symptoms like constipation. Diagnosis involves imaging tests like ultrasound, CT scans, and MRI, with surgical removal being the primary treatment option. Recurrence rates are low with complete excision of benign tumors. CONCLUSION: Retrorectal or presacral teratomas are rare tumors with vague symptoms, making diagnosis difficult. They are often detected late and require radiological assessment for surgical planning. Treatment success hinges on a coordinated effort by skilled radiologists and surgeons specializing in pelvic and oncological care to ensure favorable outcomes with lower recurrence rates and risks.