scholarly journals PEDT-3

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi11-vi11
Author(s):  
Masayuki Kanamori ◽  
Yoshiteru Shimoda ◽  
Ichiyo Shibahara ◽  
Ryuta Saito ◽  
Yukihiko Sonoda ◽  
...  
Keyword(s):  
Age Of Onset ◽  
Mature Teratoma ◽  
Germ Cell Tumors ◽  
Primary Hypothyroidism ◽  
Additional Treatment ◽  
Immature Teratoma ◽  
University Hospital ◽  
Tumor Control ◽  
Endocrine Disorders ◽  
Mixed Tumor

Abstract Background: Germ cell tumors (GCTs) containing a teratoma component is a group of diseases consisting of various pathological conditions such as mature teratoma, immature teratoma, teratoma with malignant transformation, and mixed tumor with other GCTs. There is controversy about the efficacy and safety of radiation and chemotherapy for GCTs with teratoma component other than mature teratomas. Methods: Of 212 cases of GCTs treated at Tohoku University Hospital Neurosurgery from January 1990 to March 2021. In this study, 23 histologically verified GCTs containing teratoma components were included. Pathological findings, recurrence, survival, and late complications were examined. Results: The age of onset was 2 months-21 years (median 10.5 years). Histological diagnosis was mature teratoma alone in 5 cases, mixed GCTs with mature teratoma in 11 cases, immature teratoma in 5 cases, and mixed tumor with mature teratoma and germinoma in 2 cases. Patients except mature teratoma were treated by chemotherapy alone or radiochemotherapy. During follow-up for 7–362 months (median 135 months), 3 patients relapsed. One of these patients was diagnosed with mature teratoma at the time of treatment and did not receive post-treatment, but relapsed as germinoma 21 years later. A review of pathological specimens at the time of initial onset revealed immature teratomas in addition to mature teratomas. Recurrent lesions in 3 cases were controlled by additional treatment, and no deaths due to tumor progression were observed. On the other hand, of the 18 patients who underwent radiochemotherapy, 1 developed primary hypothyroidism and 2 developed thyroid cancer and leukemia. Conclusion: GCTs with teratoma component often contain malignant histological types and require caution when making a pathological diagnosis. In these cases, tumor control can be expected by radiation or chemotherapy, but there is a risk of developing endocrine disorders and secondary tumors, and further studies are needed to optimize treatment.

scholarly journals Endocrine manifestations in a cohort of 63 adulthood and childhood onset patients with Langerhans cell histiocytosis

2019 ◽  
Vol 181 (3) ◽  
pp. 275-285 ◽  
Author(s):  
Yempabou Sagna ◽  
Carine Courtillot ◽  
Joseph Y Drabo ◽  
Abdellatif Tazi ◽  
Jean Donadieu ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Age Of Onset ◽  
Tertiary Care ◽  
Langerhans Cell ◽  
University Hospital ◽  
Endocrine Disorders ◽  
Care Hospital ◽  
Childhood Onset ◽  
Gonadotropin Deficiency

Objective Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasm which can infiltrate any organ or tissue. Endocrine involvement has mostly been described in case reports and small retrospective studies. We aimed to describe endocrine manifestations in a large cohort of adulthood onset (AO) and childhood onset (CO) patients with LCH. Design Single-center observational study conducted between January 2002 and December 2017 at Pitié-Salpêtrière University Hospital (Paris, France), a tertiary care hospital. Method Clinical, biological and morphological evaluations of pituitary, gonadal, adrenal and thyroid function evaluations performed in 63 consecutive patients with LCH (AO patients: 40, CO patients: 23). Fifty-eight patients underwent follow-up assessments. Results Complete pituitary evaluation was performed in 38/63 patients (60.3%); at least one anterior pituitary dysfunction (APD) was found in 63.2% of them. In this subgroup of patients, the most prevalent deficiencies were diabetes insipidus (DI) and GHD (55.3% each), followed by gonadotropin deficiency (34.2%) and thyrotropin deficiency (23.7%). In the subgroup of the 25 incompletely evaluated patients, we found DI in 44%, GHD in 50%, gonadotropin deficiency in 30.4% and thyrotropin deficiency in 16%. APD was more common in CO patients (P = 0.003) but was not systematically associated with DI regardless of the age of onset. Endocrine dysfunction was most often permanent; moreover, occurrence of new deficiencies has been described during follow-up. Conclusion The spectrum of endocrine disorders appears to be large in LCH (both in AO and CO patients) and should be evaluated carefully at diagnosis and during follow-up. APD was not always associated with DI.

scholarly journals Immature Teratoma and Mature Cystic Teratoma

2021 ◽  
Vol 5 (2) ◽  
pp. 139-147
Author(s):  
Nova Fenita Sari ◽  
RZ Nizar
Keyword(s):  
Germ Cell ◽  
Mature Teratoma ◽  
Relevant Literature ◽  
Primordial Germ Cells ◽  
Germ Cell Tumors ◽  
Cystic Teratoma ◽  
Residual Tumor ◽  
Scientific Paper ◽  
Immature Teratoma ◽  
Mature Cystic Teratoma

Introduction : Germ cell tumors arise from primordial germ cells and account for about 30% of all ovarian tumors. More than 95% of this group are benign dermoid cysts (mature cystic teratoma) and the remaining 5% are malignant. Ovarian teratomas represent 15% to 20% of ovarian germ cell tumors. Teratomas are classified as mature or immature and often consist of several embryological layers. While the mature type is benign, the immature type is more aggressive.Objective : Based on the above, this article will review about immature teratoma and mature cystic teratoma of the ovary. Material and methods : The method of writing this scientific paper is a literature review. The data used are sourced from relevant literature and in accordance with the topics discussed.Result : Teratomas are a common form of germ cell tumors. Teratomas are histologically defined as tumors containing tissue derived from all germ cell layers: ectoderm, mesoderm, and endoderm. Teratomas are classified as immature teratoma, mature teratoma and monodermal teratoma. Conclusion : Teratomas are usually asymptomatic and if there are symptoms, they tend to be non-specific. In patients with no residual tumor after surgery, the survival rate is 90-100%.Keywords: Teratoma, Immature Teratoma, Mature Cystic Teratoma

scholarly journals GCT-14. SECOND-LOOK SURGERY FOR INTRACRANIAL GERM CELL TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii330-iii330
Author(s):  
Hideki Ogiwara
Keyword(s):  
Germ Cell ◽  
Tumor Markers ◽  
Mature Teratoma ◽  
Germ Cell Tumors ◽  
Residual Tumor ◽  
Complete Response ◽  
Immature Teratoma ◽  
Total Resection ◽  
Mixed Germ Cell Tumor ◽  
Second Look

Abstract OBJECTIVE The authors present their experiences of second-look surgery in patients with intracranial GCTs who showed less than complete response despite normalizing or decreasing tumor markers after chemotherapy. METHODS Retrospective review of 14 patients who underwent second-look surgery for an intracranial GCT was performed. RESULTS Of 40 consecutive patients with newly diagnosed intracranial GCTs treated between August 2003 and 2019, 14 patients (35%) underwent second-look surgery. The mean age was 9.2 years. The initial diagnoses were mixed germ cell tumor in 6, immature teratoma in 4, yolk sac tumor in 2, and germinoma 2. Second-look surgery was performed after 1–3 courses of chemotherapy. Magnetic resonance imaging (MRI) at the surgery demonstrated increasing residual tumor in 8 and stable residual tumor in 6. Tumor markers were normalized in 10 and nearly-normalized in 4. Gross total resection was achieved in 12 patients and near-total resection in 2. Histopatholgy at second-look surgery revealed mature teratoma in 6, immature teratoma in 3, fibrosis with atypical cells in 2, and fibrosis in 3. Eleven patients subsequently underwent additional chemo-radiation therapy according to the initial diagnosis. All patients are alive with no evidence of recurrence with a mean follow-up of 69 months. CONCLUSIONS Second-look surgery plays an important role in the treatment of intracranial GCTs. Surgery may be encouraged at a relatively early phase after chemotherapy when the residual tumor increases or does not change the size despite normalized or nearly-normalized tumor markers in order to achieve complete resection and improve the outcome.

scholarly journals Immature teratoma

2016 ◽  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Frozen Section ◽  
Mature Teratoma ◽  
Germ Cell Tumors ◽  
Pelvic Lymphadenectomy ◽  
Immature Teratoma ◽  
Cell Tumor ◽  
Ca 125 ◽  
Grade Iii

Introduction: Immature teratoma represents 3% of all teratomas, 1 % of all ovarian cancers and 20% of malignant ovarian germ cell tumors. It is found either in pure form or as a component of a mixed germ cell tumor. It occurs essentially during the first two decades of life. According to WHO, immature teratoma is defined as a teratoma containing a variable amount of immature embryonal type neuroectodermal tissue Case: We present here a report of 23 years old unmarried female who presented with complaint of abdominal pain since 1 month and her CT scan done outside, showed fibroid uterus. She had history of typhoid fever 1 month back for which USG was done which suggested large uterine fibroid. On examination she was hemodynamically stable. On abdominal examination a non-tender supra-pubic mass of 24 weeks size with firm consistency, irregular margin was felt. On investigation CA 125 was 64.90 IU/L, LD- 223, beta HCG- 1.14. On MRI a large abdomino-pelvic lesion, likely left adnexal lesion with multiple cystic areas, with hemorrhage, with ascites and enlarged retroperitoneal lymph nodes with omental infiltration suggestive of a possibility of malignant germ cell tumor. In view of large ovarian tumor, possibly malignant decision for staging laparotomy was taken. Intra-operatively a large irregular vascular solid mass of 20 x 20 cms with bosselated appearance with few cystic lesions over it was seen, arising from left ovary and was sent for frozen section which reported malignant mature teratoma with components of immature teratoma. She underwent laparotomy with left salpingo-oophorectomy with right ovarian biopsy, omentectomy, appendectomy with B/L pelvic lymphadenectomy. Histopathology was suggestive of grade III immature teratoma. In view of grade III immature teratoma, she received chemotherapy (BEP regimen) post-operatively and is currently under follow up. Conclusion: This case reflects the importance of early diagnosis in cases of pelvic masses in young females. Fertility preservation should be considered in young women with germ cell tumors. Patients with grade II or III tumors or a mere advanced stage disease should be treated with adjuvant chemotherapy (BEP) in addition to surgery.

scholarly journals GCT-53. CASE OF INTRACRANIAL GROWING TERATOMA SYNDROME WITH DIFFICULTY IN TIMING OF RESECTION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii339-iii339
Author(s):  
Hidenobu Yoshitake ◽  
Hideo Nakamura ◽  
Yuta Hamamoto ◽  
Yusuke Otsu ◽  
Jin Kikuchi ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Endoscopic Third Ventriculostomy ◽  
Obstructive Hydrocephalus ◽  
Mature Teratoma ◽  
Timing Of Surgery ◽  
Optimal Timing ◽  
Third Ventriculostomy ◽  
Mixed Tumor ◽  
Gland Tumor ◽  
Growing Teratoma Syndrome

Abstract BACKGROUND Intracranial Growing teratoma syndrome(iGTS) is a phenomenon in which a tumor with a teratoma component grows during treatment, and its pathological tissue is often a mature teratoma. Here we report a case of iGTS in which the timing of surgery was determined by tumor markers and changes in tumor size on MRI images. CASE-REPORT: 11-year-old boy with a short stature. He developed a headache and we found a pineal gland tumor on MRI. Due to obstructive hydrocephalus, an endoscopic third ventriculostomy and biopsy were performed. The pathological diagnosis was mature teratoma, but AFP was elevated at 104.2 ng/mL. Considering NGGCT, we started chemoradiation immediately. Despite the declining AFP, it gradually increased, at which point we suspected iGTS. Resection was considered, but at some point tumor growth had stopped, so radiation therapy and a second course of ICE therapy preceded the resection. Thereafter, the tumor was completely removed, and a third course of ICE therapy was performed. DISCUSSION The onset mechanism of iGTS has not been elucidated, and its prediction is difficult. Early resection of the tumor is required, but discontinuation of radiation therapy and side effects of chemotherapy also need to be considered. In our case, resection was performed after normalization of AFP and recovery of myelosuppression. The patient followed an uneventful course, but the timing of resection was controversial. CONCLUSION We experienced a case of iGTS in NGGCT, a mixed tumor with mature teratoma. The optimal timing of the resection was discussed and literature was reviewed.

scholarly journals The Changing Paradigm for the Surgical Treatment of Large Vestibular Schwannomas

2018 ◽  
Vol 79 (S 04) ◽  
pp. S362-S370 ◽  
Author(s):  
Constantin Tuleasca ◽  
Alda Rocca ◽  
Mercy George ◽  
Etienne Pralong ◽  
Luis Schiappacasse ◽  
...  
Keyword(s):  
Functional Outcome ◽  
Residual Tumor ◽  
University Hospital ◽  
Tumor Control ◽  
Subtotal Resection ◽  
Consecutive Series ◽  
Cochlear Function ◽  
Nerve Preservation ◽  
The Past

Objective Planned subtotal resection followed by Gamma Knife surgery (GKS) in patients with large vestibular schwannoma (VS) has emerged during the past decade, with the aim of a better functional outcome for facial and cochlear function. Methods We prospectively collected patient data, surgical, and dosimetric parameters of a consecutive series of patients treated by this method at Lausanne University Hospital during the past 8 years. Results A consecutive series of 47 patients were treated between July 2010 and January 2018. The mean follow-up after surgery was 37.5 months (median: 36, range: 0.5–96). Mean presurgical tumor volume was 11.8 mL (1.47–34.9). Postoperative status showed normal facial nerve function (House–Brackmann I) in all patients. In a subgroup of 28 patients, with serviceable hearing before surgery and in which cochlear nerve preservation was attempted at surgery, 26 (92.8%) retained serviceable hearing. Nineteen had good or excellent hearing (Gardner–Robertson class 1) before surgery, and 16 (84.2%) retained it after surgery. Mean duration between surgery and GKS was 6 months (median: 5, range: 3–13.9). Mean residual volume as compared with the preoperative one at GKS was 31%. Mean marginal dose was 12 Gy (11–12). Mean follow-up after GKS was 34.4 months (6–84). Conclusion Our data show excellent results in large VS management with a combined approach of microsurgical subtotal resection and GKS on the residual tumor, with regard to the functional outcome and tumor control. Longer term follow-up is necessary to fully evaluate this approach, especially regarding tumor control.

scholarly journals Multiple sclerosis in Kenya: Demographic and clinical characteristics of a registry cohort

2021 ◽  
Vol 7 (2) ◽  
pp. 205521732110227
Author(s):  
Imran Jamal ◽  
Jasmit Shah ◽  
Peter Mativo ◽  
Juzar Hooker ◽  
Mitchell Wallin ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Symptom Onset ◽  
Clinical Characteristics ◽  
Age Of Onset ◽  
Sub Saharan Africa ◽  
University Hospital ◽  
Tertiary Referral Hospital ◽  
Female Ratio ◽  
Sub Saharan ◽  
Neuro Imaging

Background Multiple Sclerosis (MS) is the leading cause of non-traumatic neurological disability in young adults. There is limited literature regarding the burden of MS in sub-Saharan Africa (SSA). Objective To describe the demographic and clinical characteristics of patients with MS (PwMS) presenting to a tertiary referral hospital in Nairobi. Methods We conducted a retrospective descriptive study for PwMS presenting to Aga Khan University Hospital, Nairobi from 2008–2018. Results 99 cases met the diagnostic criteria for MS with a male to female ratio of 1:4. Majority (68.7%) of PwMS were indigenous Africans with a mean age of onset of 30.7 years. Mean duration from symptom onset to first neuro-imaging was 5.04 years. Only 33% of patients had sensory symptoms at onset whereas 54.5% had vitamin D deficiency/insufficiency. Majority (79.5%) had relapsing remitting MS (RRMS) and 56.6% were initiated on disease modifying therapy (DMT). Only 21.2% of patients on DMT were non-compliant. Patients with RRMS were more likely to be initiated on DMT at our hospital (p < 0.001). Conclusion Clinical characteristics of these patients largely resemble those of other SSA cohorts and African American patients. There was a delay between symptom onset and neuroimaging. There were also issues with DMT compliance.

scholarly journals Ovarian Yolk Sac Tumor: A Case Report with Review Of Literature

2016 ◽  
Vol 2 (1) ◽  
pp. 15-17
Author(s):  
A Sreehari ◽  
BM Rupakala
Keyword(s):  
Case Report ◽  
Early Adulthood ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Immature Teratoma ◽  
Yolk Sac Tumor ◽  
Histological Subtype ◽  
Review Of Literature ◽  
Yolk Sac Tumors ◽  
Ovarian Dysgerminoma

ABSTRACT A total of 3 to 5% of all ovarian malignancies include malignant ovarian germ cell tumors (MOGCTs). They are subdivided into germinomatous and non-germinomatous tumors. Common types of non-germinomatous tumors include yolk sac and immature teratoma. Ovarian yolk sac tumors (YST) are the second most frequent histological subtype of MOGCTs, after ovarian dysgerminoma. They account for 20% of MOGCTs and are frequent especially in childhood and in early adulthood. We report the case of a yolk sac tumor of the ovary in a 13yrs years old female. How to cite this article Sreehari A, Rupakala BM, Sarojamma C. Ovarian Yolk Sac Tumor: A Case Report with Review of Literature. J Med Sci 2016;2(1):15-17.

scholarly journals Adrenal Insufficiency Masquerading as Primary Hypothyroidism Following Immune Checkpoint Inhibitors Treatment

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A110-A111
Author(s):  
Michael Salim ◽  
Wafa Dawahir ◽  
Janice L Gilden ◽  
Andriy Havrylyan
Keyword(s):  
Adrenal Insufficiency ◽  
Immune Checkpoint ◽  
Immune Checkpoint Inhibitors ◽  
Cytotoxic T Lymphocyte ◽  
Checkpoint Inhibitors ◽  
Primary Hypothyroidism ◽  
Adrenal Crisis ◽  
Thyroid Peroxidase ◽  
Endocrine Disorders ◽  
Free T4

Abstract Background: Immune checkpoint inhibitors (ICIs) are novel immunotherapy agents that have been used to treat multiple advanced cancer. Even though they confer potential clinical advantages by regulating immune reactions, they have been linked with serious immune-mediated adverse events. Here we present a case of a patient who was treated with ICIs, Nivolumab (programmed death-1 inhibitor) and Ipilimumab (cytotoxic T lymphocyte antigen-4 inhibitor), and subsequently developed two concurrent immune-related endocrine disorders. Clinical Case: An 83-year-old man with advanced renal cell carcinoma presented with generalized weakness. He had finished four cycles of immunotherapy with Nivolumab and Ipilimumab, and Ipilimumab was discontinued afterward. Two days after the fifth cycle of immunotherapy with Nivolumab, he developed worsening fatigue, nausea, and anorexia. He appeared mildly volume depleted with borderline hypotensive (104/63 mmHg). The rest of the physical exam was unremarkable. Initial tests showed elevated levels of TSH (13.15 uIU/mL, ref 0.45–5.33 uIU/L), reduced levels of free T4 (&lt;0.25 ng/dL, ref 0.58–1.64 ng/dL), free T3 (1.72 pg/mL, ref 2.5–3.9 pg/mL), negative thyroglobulin antibody, and elevated levels of thyroid peroxidase antibody (429 IU/mL, ref &lt;9 IU/mL), thus suggesting primary hypothyroidism. Serum levels of sodium and potassium were unremarkable (136 meQ/L, ref 136–145 mEq/L; 3.6 meQ/L, ref 3.5–5.1 meQ/L respectively). His baseline TSH was normal three months prior to arrival (1.31 uIU/mL) and suppressed one month prior to arrival (0.01 uIU/mL). Immune-related thyroiditis with immune checkpoint inhibitors was suspected. He was given levothyroxine and observed in the hospital. After two days of hospitalization, weakness had slightly improved. However, he still had persistent nausea. He also developed low blood pressure (90/47 mmHg) and mild hyponatremia (133 mEq/L) with a normal potassium level. Further investigation showed low cortisol (1.0 ug/dL, ref 5.0–21.0), low ACTH (13 pg/mL, ref 6–50 pg/mL), cortisol level at 30 and 60 minutes post-cosyntropin stimulation test of 10.8 ug/dL (ref 13.0–30.0 ug/dL) and 14.8 ug/dL (ref 14.0–36.0 ug/dL) respectively, and negative adrenal antibodies, suggesting of secondary adrenal insufficiency due to hypophysitis. The patient was started on hydrocortisone, and his symptoms improved afterward. Conclusion: This case report highlights the common pitfall of managing immune-related endocrine disorders of ICIs. Adrenal insufficiency may present with a broad range of nonspecific symptoms, which could be attributed to hypothyroidism, underlying illness, or medications. Although a rare adverse effect, it is prudent to recognize adrenal insufficiency superimposed on primary hypothyroidism. Introducing thyroxine before replacing glucocorticoids can lead to an adrenal crisis.

scholarly journals Growing Teratoma Syndrome: Two case reports

2021 ◽  
Author(s):  
anju shrestha ◽  
Hari Dhakal ◽  
Sirish Pandey ◽  
Kapendra Amatya ◽  
Sudip Shrestha ◽  
...  
Keyword(s):  
Tumor Markers ◽  
Surgical Resection ◽  
Case Reports ◽  
Mature Teratoma ◽  
Immature Teratoma ◽  
Surgical Staging ◽  
Growing Teratoma Syndrome ◽  
Complete Surgical Resection

We present two cases of nine and twenty-seven years old girls with recurrence of immature teratoma after an incomplete surgical staging. In both cases, there were huge abdominopelvic masses despite decrease in tumor markers with chemotherapy. Complete surgical resection of these masses was done, and histopathology showed only mature teratoma.

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