Challenging gestational trophoblastic disease cases and mimics: An exemplar for the management of rare tumours.

OBJECTIVE: Rare tumour management is challenging for clinicians as evidence bases are limited and clinical trials are difficult to conduct. It is even more difficult for patients where self-reliance alone is insufficient to overcome the challenges of navigating care which is often poorly evidence based. In Ireland, a national Gestational Trophoblastic Disease (GTD) service was established as one of 3 initiatives for rare tumours by the National Cancer Control Programme. The service has a national clinical lead, a dedicated supportive nursing service and a clinical biochemistry liaison team. This study sought to assess the impact of a GTD centre using national clinical guidelines and integrating and networking with European and International GTD groups on the clinical management of challenging GTD cases and to consider the application of this model of care to other rare tumour management. STUDY DESIGN: In this article, we analyse the impact of a national GTD service on five challenging cases, and review how the service affects patient management in this rare tumour type. These cases were selected from a cohort of patients who were voluntarily registered in the service based on the diagnostic management dilemma they posed. RESULTS: Case management was impacted by the identification of GTD mimics, the provision of lifesaving treatment of metastatic choriocarcinoma with brain metastasis, networking with international colleagues, the identification of early relapse, the use of genetics to differentiate treatment pathways and prognosis, and supportive supervision of treatment courses of up to 2 years of therapy in a cohort of patients starting or completing families. CONCLUSION: The National GTD service could be an exemplar for the management of rare tumours (such as cholangiocarcinoma) in our jurisdiction which could benefit from a similar constellation of supports. Our study demonstrates the importance of a nominated national clinical lead, dedicated nurse navigator support, registration of cases and networking. The impact of our service would be greater if registration was mandatory rather than voluntary. Such a measure would also ensure equity of access for patients to the service, assist in quantifying the need for resourcing and facilitate research to improve outcomes.

Experience of women on the Irish National Gestational Trophoblastic Disease Registry.

OBJECTIVE: Gestational Trophoblastic Disease (GTD) is a rare pregnancy related disorder and the most curable of all gynaecological malignancies. GTD comprises the premalignant conditions of complete or partial hydatidiform mole known as molar pregnancy and a spectrum of malignant disorders termed gestational trophoblastic neoplasia. Clinical management and treatment in specialist centres is essential to achieve high cure rates and clinical guidelines recommend registration with a GTD centre as a minimum standard of care. National GTD registries are valuable repositories of epidemiological data and facilitate clinical audit, centralised pathology review and human chorionic gonadotropin (hCG) monitoring. This study sought the opinion of women enrolled on the Irish National GTD registry to inform future service development and establish a knowledge base for molar pregnancy in Ireland. STUDY DESIGN: A cross-sectional survey using an anonymised questionnaire was distributed by post to all women on the GTD registry. The questionnaire was designed by a multidisciplinary team and consisted of twenty-five closed-ended questions and two open-ended questions to facilitate feedback. Data collected in the survey included information on the patient experience of registration, knowledge of molar pregnancy, diagnosis at their local hospital, hCG monitoring and overall satisfaction with the service. RESULTS: The survey had a successful participation rate of 42.6% (215/504). Forty-nine percent (n = 106) of respondents rated a rapid hCG result as their top priority. Forty percent (n = 84) of women had concerns about future pregnancies but acknowledged that these were largely addressed by the GTD specialist nurses. A quarter of respondents reported that other medical professionals with whom they interacted during follow-up treatment did not understand their condition. Many women commented on the emotional stress of attending their local maternity unit for phlebotomy while dealing with pregnancy loss. CONCLUSION: This study is unique in being the first survey of women on the Irish National GTD registry. It highlights the specific needs of women with molar pregnancy in terms of psychological support, bereavement counselling and peer support groups. It reveals a knowledge gap in molar pregnancy amongst healthcare professionals which should be considered in future planning of medical and nursing curricula.

In vitro melanoma cell growth after preenucleation radiation therapy.

The in vitro efficacy of 20 Gy (2000 rad) of external beam irradiation delivered to patients with choroidal melanomas prior to enucleation was investigated in 11 patients whose tumors were grown in cell culture. Phase-contrast microscopy was used to compare growth patterns between irradiated and nonirradiated tumors. Cell types were determined by histologic stains, and electron microscopy identified intracytoplasmic melanin. Irradiated melanomas did not grow and did not attach to culture flasks, thus demonstrating that preenucleation irradiation alters the in vitro growth of melanoma cells.

Endophthalmitis after strabismus surgery with a good visual result.

A 78-year-old woman underwent recession-resection surgery for longstanding exotropia. A scleral perforation occurred at the time of surgery and was treated with transscleral retinocryopexy. Three days after surgery the patient experienced light perception vision, pain, and hypopyon. She underwent a trans pars plana vitrectomy with intravitreal and periocular antibiotics. Three months after vitrectomy, visual acuity returned to the preoperative level of 20/50. Six months after vitrectomy she underwent an uneventful cataract extraction with posterior chamber lens implant. Visual acuity 5 months after cataract surgery was 20/20.

Preservation of hematopoietic stem cells in cadaveric marrow.

CFU-C assays were performed on unselected cadavers of various postmortem intervals to determine the survival of hemopoietic stem cells in cadaveric marrows. Successful growth was achieved in eight of ten cases up to 11 hours post mortem. The two marrows in which growth was unsuccessful were from patients with underlying diseases that would have made growth impossible even if the marrow was obtained during life. Six cases in which marrow was obtained 11 to 23 hours post mortem showed no proliferative capacity. The average CFU-C in the eight cases with growth activity was 25.8 +/- 16 compared with 40.6 +/- 35 for 61 hospital patients for whom antemortem marrow cells were available for culture. These two meanings are not significantly different statistically. These findings demonstrated the persistence of hemopoietic stem cells in cadaveric marrows, showing the cadaver to be a potentially abundant source of these cells.