Challenges in the diagnosis and treatment of pure non-gestational uterine choriocarcinoma in a child: a case report.

BACKGROUND: Diagnosing non-gestational uterine choriocarcinoma in children is challenging because of its rarity and nonspecific imaging findings. Herein, we report a case of non-gestational uterine choriocarcinoma in a child, which was unexpectedly found during exploratory laparotomy and confirmed by histopathological findings. However, the tumor did not respond to chemotherapy. CASE PRESENTATION: A 4-year-old Indonesian female patient was brought into the emergency unit with chief complaint of vaginal bleeding. She had suffered from vaginal spotting 4 months before being admitted to the hospital. Physical examination revealed a distended abdomen in the left lumbar region and a palpable fixed mass with a smooth surface. Abdominal computed tomography scans revealed a large mass (10 × 6 × 12 cm) with fluid density and calcification. Thus, we suspected left ovarian teratoma. The patient's luteinizing hormone, follicle-stimulating hormone, and lactate dehydrogenase levels were 25.2 mIU/ml, 0.1 mIU/ml, and 406 U/l, respectively. According to the clinical and radiological findings, we decided to perform an exploratory laparotomy and found a tumor originating from the uterus, not the ovarium. We did not observe liver nodules and any enlargement of abdominal lymph nodes. Subsequently, we performed hysterectomy. The histopathological findings supported the diagnosis of choriocarcinoma. The patient was discharged uneventfully on postoperative day 5. Thereafter, the patient underwent nine cycles of chemotherapy, including carboplatin (600 mg/m2 IV), etoposide (120 mg/m2 IV), and bleomycin (15 mg/m2 IV). However, on the basis of the clinical findings of a palpable mass and partial intestinal obstruction, the tumor relapsed soon after the ninth cycle of chemotherapy. Currently, the patient is undergoing chemotherapy again. CONCLUSIONS: Although pure non-gestational uterine choriocarcinoma is rare, it should be considered as one of the differential diagnoses for intraabdominal tumors in a child, so as to better guide and counsel families regarding the surgical plan and prognosis, respectively. In the present case, the patient's response to chemotherapy was poor, implying that the treatment of non-gestational choriocarcinoma is still challenging, particularly in the pediatric population.

Primary non-gestational mediastinal choriocarcinoma metastatic to the brainstem.

Choriocarcinoma is a highly malignant tumour emerging from the syncytiotrophoblast divided into gestational and non-gestational presentations. Primary choriocarcinoma of the mediastinum is rare. Metastases to the brain often occur; however, brainstem involvement has not been reported for non-gestational choriocarcinoma. We described a middle-aged man who developed a complete left oculomotor nerve paralysis secondary to a brainstem tumour at the midbrain. The workup for the primary source of the brainstem tumour included a chest CT scan, which revealed a mediastinal mass. A mediastinal mass needle biopsy confirmed the diagnosis of primary mediastinal choriocarcinoma. Despite aggressive chemotherapy, the patient died 6 months after the initial presentation from neurological complications and multiorgan failure.

Clinical Experience of Male Primary Choriocarcinoma at the Samsung Medical Center.

PURPOSE: The objective of this study was to describe and analyze the clinicopathological features of primary choriocarcinoma (PCC) observed in male patients treated at the Samsung Medical Center between 1996 and 2020. MATERIALS AND METHODS: We reviewed the clinical records of 14 male patients with PCC retrospectively to assess their demographic, histological, and clinical characteristics at the time of diagnosis as well as identify the treatment outcomes. RESULTS: The median age of the patients was 33 years. The primary tumor site was the testicles in seven cases (50%), the mediastinum in six cases (43%), and the brain in one case (7%). The most common metastatic site was the lungs (79%), followed by the brain (43%). All patients with PCC received cytotoxic chemotherapy. Twelve patients had records of their response to cytotoxic chemotherapy; of these 12 patients, eight (8/12, 67%) achieved an objective response, and four (4/12, 33%) achieved stable disease response as the best response during chemotherapy. CONCLUSION: It is known that most male PCC patients eventually develop resistance to cytotoxic chemotherapy and die. Factors such as poor response to chemotherapy, high disease burden, brain metastasis, and hemoptysis at the time of diagnosis are associated with shorter survival time in male PCC patients. Programmed death-1/programmed death-ligand 1 blockade therapy can be a salvage treatment for chemotherapy-resistant male PCC patients.

Short tandem repeat analysis for confirmation of uterine non-gestational choriocarcinoma in a postmenopausal Taiwanese woman.

RATIONALE: Rare uterine choriocarcinoma can be differentiated gestational from nongestational choriocarcinoma by using short tandem repeats (STRs). PATIENT CONCERNS: A 56-year-old Taiwanese woman underwent staging surgery because of suspicion of high-grade endometrial cancer. The pathology-confirmed uterine tumor with syncytiotrophoblasts and decidual change of the endometrium was harvested. DIAGNOSIS: Uterine nongestational choriocarcinoma. INTERVENTIONS: The tumor specimen, the patient's blood, and her husband's blood were drawn for STRs analysis using polymerase chain reaction amplification kit. The genotype of the tumor cells was solely maternal and made the diagnosis of uterine nongestational choriocarcinoma. OUTCOME: Adjuvant chemotherapy with etoposide, methotrexate, actinomycin D, cyclophosphamide, vincristine regimen achieved good response in the patient. The patient is now recurrence-free for 12 months. LESSONS: STRs aid precise classification of rare choriocarcinoma. We encourage using the method to analyze suspicious choriocarcinoma.

Response to anti-programmed cell death protein-1 antibodies in men treated for platinum refractory germ cell cancer relapsed after high-dose chemotherapy and stem cell transplantation.

INTRODUCTION: Treatment options for patients with platinum refractory metastatic germ cell tumours (GCT) relapsing after high-dose chemotherapy and autologous stem cell transplantation are limited and survival is poor. Antibodies directed against programmed cell death protein-1 (PD-1) and programmed cell death ligand-1 (PD-L1) are currently assessed within clinical trials. We present updated data on our experience with checkpoint inhibitors as a compassionate use off-label treatment attempt for highly-pretreated patients with GCT and provide an overview of the current literature on PD-L1 expression in this rare tumour entity. PATIENTS AND METHODS: We analysed all patients with platinum refractory GCT treated with checkpoint inhibitors at our institutions between 2015 and 2017. Data were retrieved retrospectively from the patient charts. RESULTS: Seven patients were treated with nivolumab or pembrolizumab. Four patients received single-dose treatment and died shortly afterwards due to tumour progression; the remaining three patients received treatment for at least 6 months. No significant treatment toxicity was observed. Long-term tumour response was achieved in two of the three patients, both of them highly positive for PD-L1 staining. INTERPRETATION: We consider checkpoint inhibition to be efficient in carefully selected patients with platinum refractory GCT. However, predictive markers associated with tumour response are not yet known and larger prospective clinical trials are warranted.

Primary mediastinal choriocarcinoma with brain metastasis in a female patient.

Nongestational choriocarcinoma is very rare and carries a poor prognosis in female patients. In this report, the authors present a case of nongestational choriocarcinoma with brain metastasis in a female. A 58-year-old female with intermittent back pain was referred to a private hospital. On examination, a mediastinal tumor and a pancreatic tumor were detected. Endoscopic ultrasound-guided fine needle aspiration biopsy of the tumor was performed for histological evaluation. Pathological diagnosis was difficult because only a small amount of tissue was collected. Head MRI showed multiple metastatic tumors in the brain. The patient was diagnosed with primary mediastinal choriocarcinoma with brain metastasis. She was treated with one course of an etoposide, methotrexate, dactinomycin, cyclophosphamide, and vincristine regimen, but her general condition gradually deteriorated, and she died on day 41. Nongestational choriocarcinoma is drug resistant, whereas gestational choriocarcinoma has better chemotherapeutic sensitivity.

Pure non-gestational choriocarcinoma arising in the ovary.

Non-gestational choriocarcinoma (NGCO) is a rare primary ovarian cancer with poor prognosis. It is important to distinguish it from gestational ovarian choriocarcinoma (GCO), because there are different treatment options. However, it is difficult to distinguish the two types by routine histologic, ultrastructural, or immunohistochemical examination. The authors present NGCO in a 41-year-old woman, which was confirmed by DNA polymorphism analysis. All tested microsatellite markers had identical DNA profiles with the same allelic sizes between tumor and normal myometrium of the patient, indicating that both tissues originated from the same person. The results confirmed that the tumor was non-gestational in origin. Although the tumor was large, the authors performed hand- assisted laparoscopic surgical (HALS) staging. After three cycles of combination chemotherapy and surgery, the patient has not had any evidence of disease 48 months after treatment. This case demonstrates the usefulness of HALS staging and DNA polymorphism analysis in NGCO.

[Modified BEP regimen leads to abatement of choriocarcinoma syndrome in a patient with extra gonadal germ cell tumor : a case report].

We present a case study of a 46-year-old man with extra gonadal germ cell tumor with multiple lung metastases and very high levels (324,100 mIU/ml) of the tumor marker human chorionic gonadotropin (hCG). He underwent chemotherapy with VP-16, ifosfamide and cisplatinum regimen, but on day 2, he noticed strong dyspnea. A chest X-ray showed bilateral infiltration of the lungs, and he was diagnosed with acute respiratory distress syndrome (ARDS) from choriocarcinoma syndrome. After ARDS improved, he underwent modified bleomycin, VP-16 and cisplatinum for induction therapy again. After salvage chemotherapies, levels of the tumor marker hCG decreased to normal levels, and retroperitoneal lymph node dissection and left lung wedge resection were performed to confirm pathological complete remission. No obvious recurrence, as shown by tumor markers and imaging studies, has been observed for 17 months after the treatments.

The encouraging prognosis of nongestational ovarian choriocarcinoma with lung metastases.

OBJECTIVE: To study nongestational ovarian choriocarcinoma (NGOC) with lung metastases: its early diagnosis, optimal therapeutic method, and prognosis. STUDY DESIGN: Twelve cases of NGOC with lung metastases treated in Peking Union Medical College Hospital from 1982-2011 were analyzed retrospectively. The 12 cases included 9 pure NGOCs and 3 mixed with other germ cell tumors (mature teratoma, endodermal sinus tumor and embryonal carcinoma components, and dysgerminoma, respectively). Chemotherapy was given in all 12 cases, mainly including EMA/CO, BEP, and RESULTS: The median age for the cases was 23.9 years. Abdominal pain was the most common symptom (7/12). Follow-up was available for 11 cases, ranging from 17-174 months (median, 86.6 months). Of those, only 1 patient died of the disease, at 42 months from the disease onset. The other patient for whom follow-up was not available gave up treatment due to chemoresistance and disease progression. An overall sustained remission had been achieved in 10 cases (83.3%). CONCLUSION: Surgery combined with the appropriate chemotherapy regimen can improve therapeutic efficacy and survival in the treatment of NGOC with lung metastasis, even in recurrent or chemorefractory cases. Commencement of EMA/CO chemotherapy, which seems to be associated with better prognosis, should be considered as a good choice of treatment. Conservative surgery is acceptable for young patients desiring to preserve fertility.

Pure nongestational choriocarcinoma of the ovary: a case report.

Pure ovarian choriocarcinoma can be gestational or nongestational in origin. Nongestational choriocarcinoma of the ovary is extremely rare, and its diagnosis is very difficult during the reproductive years. We present a case of a 33-year-old woman diagnosed with pure nongestational ovarian choriocarcinoma. Following surgery, multiple courses of a chemotherapy regimen of etoposide, methotrexate, and actinomycin-D (EMA) were effective.

Pathological complete response and two-year disease-free survival in a primary gastric choriocarcinoma patient with advanced liver metastases treated with germ cell tumor-based chemotherapy: a case report.

Choriocarcinoma is an early metastasizing and highly invasive tumor and characterized as a high-level human chorionic gonadotropin-secreting tumor. It normally arises in the gestational trophoblast, gonads and much less frequently in the stomach. Primary gastric choriocarcinoma appears to have a poor prognosis; especially with liver metastasis, the survival period is expected to be <1 month. This unfavorable clinical outcome is partly due to the lack of defined chemotherapy against primary gastric choriocarcinoma. We herein report a case of a 68-year-old male primary gastric choriocarcinoma patient with advanced liver metastases in which germ cell tumor-based chemotherapy achieved a pathological complete response and 2-year disease-free survival.

A rare case of bilateral massive hemothorax from spontaneous rupture of a primary mediastinal mixed germ cell tumor.

Spontaneous rupture of a mediastinal germ cell tumor, while rare, is always accompanied by bleeding. In this report, we describe a case of a primary mediastinal mixed germ cell tumor that presented with bilateral massive hemothorax and hemorrhagic shock. An urgent thoracotomy, which was performed to control bleeding, confirmed bilateral hemothorax secondary to a ruptured mediastinal tumor. Pathologic diagnosis revealed the mediastinal tumor to be mixed choriocarcinoma and immature teratoma, with lung metastatic choriocarcinoma. The patient recovered well from the operation and received salvage chemotherapy. Two years after diagnosis, the patient remains in remission with no evidence of disease.

Unusual presentation of primary gastric choriocarcinoma in a 24-year-old female patient.

We report a case of a young female patient presenting with a high serum beta-HCG levels, amenorrhea, nausea and anemia which mimicked pregnancy followed by upper gastrointestinal bleeding. A gastric tumor was shown on endoscopy. Histopathologic evaluation revealed Primary Gastric Choriocarcinoma (PGC). The patient was treated with three cycles of standard nongestational choriocarcinoma chemotherapy. Tumor persistence was evidenced by CT Scans and high serum beta-HCG levels. The patient died approximately six months after diagnosis. Our case report suggest that PGC is a highly aggressive tumor that is often associated with liver and lungs metastasis without evidence of pelvic organ abnormality and is associated with some hormonal effects, such as amenorrhea, anemia, nausea and vomiting mimicking pregnancy in young adult female

Good prognosis for primary ovarian pure nongestational choriocarcinoma using the EMA/CO regime.

Nongestational choriocarcinoma of the ovary is a rare germ cell tumor with a worse prognosis than gestational choriocarcinoma. In this report we present a case of nongestational chroriocarcinoma where the EMA/CO regime was applied. The clinical features, management, and outcome are discussed.

[Multiple hepatic metastases due to germ cell tumor on initial clinical presentation].

A 31-year-old man presented with abdominal pain. Abdominal computed tomography (CT) demonstrated heterogeneously enhanced liver masses and chest CT revealed an anterior abnormal mass. He was admitted for further examination and treatment. The patient's serum beta-HCG level was markedly elevated (2,300ng/ml) and liver biopsy revealed the presence of choriocarcinoma and positive immunostaining for HCG. The patient was suspected to have combined germ cell tumor in the mediastinum with multiple liver metastases. He was treated with 8 cycles of BEP therapy (cisplatin. etoposide, bleomycin) and the beta-HCG level was normalized. We report a case of germ cell tumor with multiple and diffuse hypervascular masses in the liver as the initial clinical manifestation.

Primary non-gestational uterine cervical choriocarcinoma with metaplastic transformation from squamous cells.

BACKGROUND: Primary non-gestational uterine cervical choriocarcinoma is very unusual and although it has been hypothesized that it can arise by metaplastic transformation of cervical epithelium, solid evidence has been lacking. CASE: Primary non-gestational uterine cervical choriocarcinoma was diagnosed in a 47-year-old, woman undergoing tubal resection 17 years previously. A histologically- and immunohistochemically-confirmed, non-gestational cervical choriocarcinoma could be diagnosed in which there was metaplastic transformation from squamous cells . The patient underwent 5 courses of an actinomycin-D chemotherapeutic regimen and radical hysterectomy with bilateral pelvic lymphadenectomy. CONCLUSION: Primary non-gestational uterine cervical choriocarcinoma may indeed arise from metaplastic transformation of epithelial tissue.