Cancer in pregnancy: breast cancer.

Breast cancer is the most common malignancy in women, and for women under 40, it is the leading cause of cancer-related deaths. A specific type of breast cancer is pregnancy-associated breast cancer, which is diagnosed during pregnancy, the first-year postpartum, or during lactation. Pregnancy-associated breast cancer is seen in 3/1000 pregnancies and is increasing in incidence as women delay pregnancy. This type of breast cancer is more aggressive, and not infrequently, there is a delay in diagnosis attributed to physiologic changes that occur during pregnancy and a lack of awareness among physicians. In this review, we discuss the demographics of pregnancy-associated breast cancer, provide differential considerations, and illustrate the multimodality imaging features to bring attention to the radiologist about this aggressive form of breast cancer.

The trend in cesarean myomectomies and the risk of obstetrical complications in Korea.

BACKGROUND: To evaluate pregnancy outcomes and the risk of adverse obstetrical outcomes of cesarean myomectomy (CM) compared with cesarean section (CS) only, and to investigate the trend of surgeons in choosing CM. METHODS: A retrospective cohort study was performed on all patients who underwent CS complicated by leiomyoma at two university hospitals between January 2010 and May 2020. All patients were categorized into the CM (341 women) or CS-only (438 women) group. We analyzed the demographic factors, obstetric factors, surgical outcomes, and possible risk factors for adverse outcomes between the two groups. RESULTS: Women who underwent CS only were significantly more likely to have a previous myomectomy and multiple leiomyoma history than women who underwent CM. The gestational age at delivery and pregnancy complications were significantly higher in the CS-only group. The mean size of the leiomyomas was larger in the CM group than in the CS-only group (5.8 ± 3.2 cm vs. 5.2 ± 3.1 cm, P = 0.005). The operation time and history of previous CS and preterm labor were higher in the CM group. The leiomyoma types differed between the two groups. The subserosal type was the most common in the CM group (48.7%), and the intramural type was the most common in the CS-only group. Patients in the CM group had fewer than three leiomyomas than those in the CS-only group. Preterm labor and abnormal presentation were relatively higher in the CM group than in the CS-only group, concerning leiomyoma presence. There were no significant differences in the preoperative and postoperative hemoglobin levels. The size of the leiomyoma (odds ratio [OR] = 1.162; 95% confidence interval [CI]: 1.07-1.25; P < 0.001) and operation time > 60 min (OR = 2.461; 95% CI: 1.45-4.15) were significant independent predictors of adverse outcomes after CM. CONCLUSIONS: CM should be considered a reliable and safe approach to prevent the need for another surgery for remnant leiomyoma. Herein, surgeons performed CM when uterine leiomyomas were large, of the subserosal type, or few. Standardized treatment guidelines for myomectomy during CSs in pregnant women with uterine fibroids should be established.

The risk of developing a meningioma during and after pregnancy.

Pregnancy has been associated with diagnosis or growth of meningiomas in several case reports, which has led to the hypothesis that pregnancy may be a risk factor for meningiomas. The aim of this study was to test this hypothesis in a large population-based cohort study. Women born in Sweden 1958-2000 (N = 2,204,126) were identified and matched with the Medical Birth Register and the Cancer Register. The expected number of meningioma cases and risk ratios were calculated for parous and nulliparous women and compared to the observed number of cases. Compared to parous women, meningiomas were more common among nulliparous (SIR = 1.73; 95% CI 1.52-1.95). The number of meningioma cases detected during pregnancy was lower than the expected (SIR = 0.40; 95% CI 0.20-0.72). Moreover, no increased risk was found in the first-year post-partum (SIR = 1.04; 95% CI 0.74-1.41). Contrary to our hypothesis, there was no increased risk for diagnosing a meningioma during pregnancy or 1-year post-partum. A lower detection rate during pregnancy, may reflect under-utilization of diagnostic procedures, but the actual number of meningiomas was homogenously lower among parous than nulliparous women throughout the study period, indicating that pregnancy is not a risk factor for meningioma.

Outcomes of Patients with Macroprolactinoma Desiring Pregnancy: Follow-Up to 23 Years from a Single Center.

Macroprolactinomas are rarely seen in women, and pregnancy is a risk factor for tumor growth. More studies are needed to determine appropriate management for macroprolactinoma and pregnancy. The aim of our study is to evaluate effects of treatment with dopamine agonists on macroadenoma before and during pregnancy, safety of dopamine agonists on fetus, post-pregnancy prognosis and long-term results. This is a single center retrospective study. Thirty-four pregnancies occurred in 21 patients under medical therapy. Prolactin levels, treatment results, tumor diameter changes, maternal-fetal outcomes, and disease activity were evaluated. The median tumor size at the time of diagnosis was 15 mm (10-28). Residual adenoma diameter was smaller in those receiving medical therapy longer than one year till the conception (p=0.047). Treatment was discontinued in 28 pregnancies after pregnancy confirmation, and 6 patients were exposed to bromocriptine throughout pregnancy. There was no symptomatic tumor growth during gestation. Among 27 live births, none of the fetuses developed neonatal malformation except for a case of Down syndrome. While early remission rate after pregnancy was 9.5%, this rate reached 33.3% at last follow-up visit. Lowered PRL levels at postpartum period (p=0.040), smaller tumor size at last follow-up visit (p=0.030), and total disappearance of tumor (p=0.026) were the contributor factors for remission. Use of dopamine agonist over one year may reduce the risk of symptomatic tumor growth during pregnancy in patients without invasive or large macroprolactinoma before pregnancy. Exposure to dopamine agonists seems generally safe for the fetus.

Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts.

Pregnancy in women with lymphangioleiomyomatosis (LAM) has been associated with increased complications and worsening lung function although objective data to advise patients are not available. We assessed lung function and CT scans before and after pregnancy in 16 women with LAM. During the pregnancy, pneumothorax was frequent and mean forced expiratory volume in 1 s (FEV1) fell from 77%±19% prepregnancy to 64%±25% predicted and DLCO from 66±26 to 57±26 (both p<0.01). After pregnancy, rates of FEV1 decline were high and 10 patients required sirolimus. Women with LAM, especially with moderate or advanced disease should be counselled regarding adverse events and loss of lung function during the pregnancy.

Perinatal outcome of pregnancies complicated by placental chorioangioma: systematic review and meta-analysis.

OBJECTIVE: To report the perinatal outcome of singleton pregnancies complicated by placental chorioangioma diagnosed on prenatal ultrasound. METHODS: MEDLINE, EMBASE, CINAHL and ClinicalTrials.gov databases were searched for studies reporting the outcome of pregnancies complicated by placental chorioangioma. Inclusion criteria were singleton pregnancy diagnosed with placental chorioangioma on prenatal ultrasound, with no other associated structural anomaly. The primary outcome was perinatal mortality. Secondary outcomes included associated non-structural anomalies detected on prenatal ultrasound (including fetal hydrops, anemia, polyhydramnios, signs of hyperdynamic circulation and small-for-gestational-age (SGA) fetus), SGA at birth, composite neonatal morbidity and preterm birth. Outcome was assessed separately in pregnancies undergoing and those not undergoing fetal therapy. Subanalyses were performed according to the presence of hydrops and the size of the tumor in all pregnancies diagnosed with chorioangioma. Random-effects meta-analyses of proportions were used to analyze the data. RESULTS: Twenty-eight studies (161 pregnancies) were included. In pregnancies complicated by chorioangioma that did not undergo intervention, intrauterine death occurred in 8.2% (95% CI, 3.8-15.0%), while neonatal death and perinatal death occurred in 3.8% (95% CI, 1.0-8.1%) and 11.1% (95% CI, 5.0-19.4%), respectively. SGA at birth was present in 24.0% (95% CI, 13.5-36.5%) of cases, while preterm birth < 37 weeks complicated 34.1% (95% CI, 21.1-48.3%) of pregnancies. Composite neonatal morbidity occurred in 12.0% (95% CI, 4.5-22.3%) of cases. On ultrasound, signs of fetal hyperdynamic circulation were present in 21.0% (95% CI, 9.6-35.3%) of cases, while peak systolic velocity in the fetal middle cerebral artery was increased in 20.6% (95% CI, 10.9-32.3%). Subanalysis according to the size of chorioangioma, including both pregnancies that did and those that did not undergo intervention, showed a progressive increase in the occurrence of most of the outcomes explored with increasing size of the tumor. Furthermore, the prevalence of adverse perinatal outcome was high in pregnancies complicated by chorioangioma presenting with fetal hydrops. There was no randomized controlled trial comparing intervention vs expectant management in pregnancies complicated by chorioangioma with signs of fetal compromise (hydrops or hyperdynamic circulation). Overall, perinatal mortality occurred in 31.2% (95% CI, 18.1-46.1%) of fetuses undergoing in-utero therapy, and 57.3% (95% CI, 39.2-74.4%) had resolution of hydrops or hyperdynamic circulation after treatment. CONCLUSIONS: Placental chorioangioma is associated with adverse perinatal outcome. The size of the mass and presence of fetal hydrops are likely to be the main determinants of perinatal outcome in affected pregnancies. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

[Familial adenomatous polyposis, desmoid tumors and Gardner syndrome]. / Polypose adénomateuse familiale, tumeurs desmoïdes et syndrome de Gardner.

About 15 % of patients with familial adenomatous polyposis "PAF" develop one or more desmoid tumors in their lifetime. These are benign mesenchymal tumors with local aggressivity but with no potential for metastases. Most of the desmoids tumors result from a sporadic genetic anomaly in the ß catenin gene. When related to familial adenomatous polyposis or "PAF", this mutation is not present, and the patients must be sent in genetic counselling. The PAF is a dominant autosomic illness related to a germinal mutation in the APC gene. Sometimes, these tumors can be the first manifestation of the illness. The diagnosis in a context of PAF can be easily done by imaging, but a pathological confirmation is needed. These tumors raise a therapeutic problem because of their heterogeneity and the absence of predictive biomarkers along illness evolution. The identification of prognostic biological and clinical factors would make easier the selection of patients requiring first-line treatment, as spontaneous remissions have also been observed in patients with FAP whom which an active surveillance could also be a valid therapeutic option. The particularity of desmoids tumors associated to PAF lies in their predominantly intra-abdominal location and the risk of complication. In the last ten years, surgery has largely given way to conservative treatments such as chemotherapy and more recently to tyrosine kinase inhibitors that have shown their efficacy with a significant improvement in progression-free survival of patients.

Fertility preservation with successful pregnancy outcome in a patient with transplanted heart and non-Hodgkin's lymphoma - a case report.

BACKGROUND: Fertility preservation must be discussed with reproductive age women before cancer treatment. Heart transplantation raises complex issues in pregnancy. Pregnancy in a heart transplant woman after pelvic irradiation involves close multidisciplinary follow-up to avoid complications in the mother and the foetus. We report the first live birth in a heart transplant woman after pelvic irradiation, chemotherapy and fertility preservation. CASE PRESENTATION: A 36-year-old heart transplant woman with pelvic non-Hodgkin lymphoma spared her fertility, with cryopreservation of oocytes and embryos, before chemotherapy and pelvic irradiation. After multidisciplinary discussion and pre-conception evaluation, pregnancy was achieved. A close follow-up by a multidisciplinary team allowed a normal pregnancy without maternal or foetal complications and the delivery of a healthy infant. CONCLUSIONS: Achieving pregnancy in heart transplant women with iatrogenic ovarian failure after oncologic treatment including pelvic irradiation is possible and can be successful. Careful and close surveillance by a multidisciplinary team is mandatory due to increased risk of maternal and foetal complications.

A partial molar pregnancy associated with a fetus with intrauterine growth restriction delivered at 31 weeks: a case report.

INTRODUCTION: Molar pregnancies belong to a group of diseases classified as gestational trophoblastic diseases, which result from an altered fertilization. Partial molar pregnancy with a live fetus is a very rare condition, occurring in 0.005 to 0.01% of all pregnancies; it presents a challenging diagnosis, especially when clinical signs are almost completely absent. CASE PRESENTATION: Here we report a rare case of partial molar pregnancy in which a normal-appearing male fetus with diploid karyotype was delivered at 31 weeks gestation by a 37-year-old white woman. The pregnancy was characterized by an episode of threatened abortion in the first trimester and an ultrasonographic diagnosis of intrauterine growth restriction. Our patient did not report any suspicious symptoms for trophoblastic disease. Due to impaired umbilical artery velocimetry with an absence of the diastolic phase, she underwent an emergency caesarean section at 31 weeks and delivered an 880 g male baby. The male baby was normal without any complications at 3-month and 12-month follow-up and the mother had no evidence of recurrence after 3 and 12 months of follow-up. Pathological examination of the placenta showed changes of partial hydatidiform mole. CONCLUSION: Partial molar pregnancy with a live fetus is a very rare condition that presents a challenging diagnosis. Recognizing it is of primary importance for patient care and the placenta should always be investigated at birth, especially in a newborn with intrauterine growth restriction.

A successful pregnancy in a patient with secondary hypertension caused by adrenal adenoma: a case report.

BACKGROUND: Secondary hypertension is a rare complication in pregnancy that causes poor outcomes, such as preeclampsia, premature delivery, intrauterine growth retardation, stillbirths, spontaneous abortion or intrauterine death. Cushing's disease caused by an adrenal adenoma is rare during pregnancy and may be overlooked by obstetricians and physicians, but can lead to hypertension, diabetes mellitus and an increased risk of fetal and maternal morbidity. Approximately 200 cases have been reported in the literature. Here, we report the successful management of a pregnant patient with Cushing's syndrome due to an adrenal adenoma. CASE PRESENTATION: The 35-year-old Chinese female had no individual or family medical history of hypertension, and did not exhibit chronic kidney disease, diabetes mellitus, autoimmune and common endocrine diseases. Her blood pressure was elevated from the 16th week of gestation and was not controlled by 30 mg nifedipine twice a day. Examination in our department revealed her 24 h urinary free cortisol (24 h UFC) level was 1684.3 µg/24 h (normal range: 20.26-127.55 µg/24 h) and plasma adrenocorticotropic hormone was < 1.00 ng/L in three independent measurements (normal range: 5-78 ng/L). Ultrasonography demonstrated a mass (2.9 cm × 2.8 cm) in the right side of the adrenal gland. Magnetic resonance imaging without contrast showed a 3.2 cm diameter mass in the right-side of the adrenal gland. Other medical tests were normal. Laparoscopic adrenalectomy was performed at the 26th week of gestation by a urological surgeon in the West China Hospital. Histopathology revealed an adrenocortical adenoma. After surgery, the patient accepted glucocorticoid replacement therapy. The remaining trimester continued without complication and her blood pressure was normal at the 32nd week of gestation without antihypertensive therapy. The patient gave birth to a healthy boy at the 40th week of gestation. CONCLUSIONS: Cushing's syndrome caused by adrenal adenoma is rare during pregnancy. This unique case suggested that analysis of the UFC level and circadian rhythm of plasma cortisol provides a suitable strategy to diagnose Cushing's syndrome during pregnancy. Laparoscopic surgical resection in the second trimester provides a reasonable approach to treat pregnant patients exhibiting Cushing's syndrome caused by an adrenal adenoma.

Fatal Intracranial Hemorrhage in a Young Pregnant Patient with Acute Promyelocytic Leukemia.

Acute promyelocytic leukemia during pregnancy is associated with a high risk of fetal and obstetrical complications. Although the prognosis of acute promyelocytic leukemia is favorable due to disease-specific drugs, such as all-trans retinoic acid, early death due to fatal intracranial hemorrhage has been observed in some cases. In this study, we present a case of catastrophic intracranial hemorrhage in a young pregnant patient with the finding of leukemic involvement of the placenta. To our knowledge, this is the first confirmed case of acute promyelocytic leukemia involving the placenta. The clinical history, pertinent histological findings, and clinical outcomes will be discussed.

Accelerated growth of hemangioblastoma in pregnancy: the role of proangiogenic factors and upregulation of hypoxia-inducible factor (HIF) in a non-oxygen-dependent pathway.

Hemangioblastomas (HBs) are benign, highly vascular tumors, often characterized by loss of function of the von Hippel-Lindau (vHL) gene. They are the most common central nervous system tumor observed in vHL syndrome. Loss of function of the vHL gene creates a "pseudo-hypoxic" state, causing overactivation of hypoxia-inducible factor (HIF) and vascular endothelial growth factor (VEGF)-related pathways. In some cases, HBs can rapidly increase in size during pregnancy to then present acutely, which most frequently occurs after the 20th gestational week. These changes in size usually occur from enlargement of the cystic component of the HB. Due to their preferred location in the posterior fossa near critical structures as well as along the spinal cord, such cases can present with severe neurological deficits, requiring urgent surgical intervention in a multidisciplinary setting. However, the reasons for this acute flare-up during pregnancy remain poorly understood, as are the reasons why this occurs in only a subset of tumors. Unveiling the etiology for this clinical scenario can affect the treatment of HBs, as it will contribute to the understanding of the pathophysiology of such a transformation from a quiescent lesion to a symptomatic one, not only in the setting of pregnancy. Identifying the correct triggers and the conditions initiating and mediating this switch will enable us to develop preventive medications which should allow us to keep the tumor in its quiescent phase. In this pathophysiological review, we investigate the association between HB growth and pregnancy based on an analysis > 40 such published cases. We suggest that the proangiogenic state of pregnancy is the leading etiology for this striking association, and to support the argument, we discuss its potential impact on HIF overexpression in a non-hypoxic manner through activation of the PI3K/Akt/mTOR pathway by proangiogenic factors. Specifically, we discuss the involvement of placental growth factor (PlGF) and its receptor vascular endothelial growth factor receptor 1 (VEGFR-1) in various pathologic processes that can lead to the formation and growth of peritumoral edema and cysts, which are the primary causes for the development of any symptoms in HB. Both PlGF and VEGFR-1 are expressed at increased levels during pregnancy, and both have been reported as part of various pathological processes, including angiogenesis and tumorigenesis. The unique feature that both do essentially not show any significant negative impact on regular physiological processes makes them attractive therapeutic targets since very little side effects are expected. Further research into the effects of anti-PlGF or anti-VEGFR-1 therapy in HB is therefore recommended.

Characteristics and diagnosis of pregnancy and lactation associated breast cancer: Analysis of a self-reported regional registry.

BACKGROUND: Pregnancy-associated breast cancer (PABC) is the most common malignancy in pregnancy. However due to its infrequent occurrence, PABC continues to be poorly understood. METHODS: We performed a retrospective study using self-reported data from 1079 eligible women in a regional breast cancer registry. RESULTS: The PABC cases were more likely than non-PABCs to be younger than age 35 and have nodal involvement at diagnosis. Despite diagnosis at a young age, there was not an association between PABC and family history. For method of diagnosis, PABC was found on self-exam, while non-PABCs were found on mammography. CONCLUSION: In conclusion, PABC is rarely detected by mammography and diagnosis is highly dependent on detection during self-breast exam. Women who are or recently were pregnant should be encouraged to perform regular self-breast exams to report any changes for further evaluation. Patient and clinician education regarding risk and realities of PABC is essential.

Retroperitoneal ganglioneuroma combined with scoliosis: A case report and literature review.

RATIONALE: Ganglioneuroma (GN) is a rare tumour arising from the sympathetic nervous system. GN is constantly asymptomatic, easily ignored and likely damages other organs during tumour progression. PATIENT CONCERNS: The case report involved a 21-year-old girl who was admitted to a hospital because of a computed tomography result after her pregnancy examination showed retroperitoneal tumour and scoliosis. The scoliosis was considered as a tumour complication. DIAGNOSES: The tumour was finally diagnosed as GN by pathological examination. INTERVENTIONS: We carried out surgical treatment and performed a pathological examination on postoperative tumour specimens. OUTCOMES: The patient was followed up for 19 months and did not show tumour recurrence. However, the condition of the scoliosis did not improve. LESSONS: This paper reports a case of GN with scoliosis at the same time. GN is a benign tumour consisting of cells with a special origin. GN grows extensively and leads to different complications. Presently, pathological examination after an operation is the only approach to formulate an exact diagnosis. We should consider the possibility of retroperitoneal tumour, especially GN, if a patient suffers from scoliosis with an unknown cause. Thus, CT and MRI are needed to provide additional information that would help formulate a diagnosis.

Pregnancy and gastric cancer: diagnostic and treatment dilemma.

Gastric cancer in pregnancy is an extremely rare entity. The incidence of gastric cancer is more in elderly patients and higher in the male population. Gastric cancer symptoms can be nausea, vomiting and epigastric discomfort which is similar to early pregnancy symptoms. This leads to a misinterpretation and delay in the diagnosis during pregnancy. Gastric cancer in pregnancy is associated with poor prognosis because of its delayed diagnosis at an advance stage. We present our patient here, with this rare clinical diagnosis and with no suspicion in a young healthy woman.