Placental site trophoblastic tumour: the rarest subtype of gestational trophoblastic disease.

Placental site trophoblastic tumour (PSTT) is a very rare form of gestational trophoblastic disease that grows slowly, secretes low levels of beta-subunit of human chorionic gonadotropin (ß-hCG), presents late-onset metastatic potential and is resistant to several chemotherapy regimens. Here, we report a case of PSTT in a 36-year-old woman who presented with amenorrhea and persistently elevated serum level of ß-hCG after a miscarriage. Transvaginal ultrasound revealed a hypovascular ill-defined solid lesion of the uterine fundus and MRI showed a tumour infiltrating the external myometrium with discrete early enhancement and signal restriction on diffusion-weighted imaging. PSTT was suspected, and after endometrial biopsy by hysteroscopy and posterior hysterectomy, microscopic examination allowed the final diagnosis. The level of ß-hCG dropped significantly in about a month after surgical treatment. Due to the rarity of PSTT, reporting new cases is crucial to improve the diagnosis and managing of these patients.

CARE-Placental site trophoblastic tumor presenting with thrombotic microangiopathy: A case report.

RATIONALE: Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic neoplasm (GTN). PATIENT CONCERNS: A 28-year-old Chinese female with initial presentation of thrombotic microangiopathy (TMA) with symptoms of edema of the lower extremities and eyelid, thrombocytopenia and anemia. DIAGNOSES: The primary diagnosis was chronic glomerulonephritis according to the related laboratory tests and clinical symptoms. INTERVENTIONS: A total abdominal hysterectomy with a bilateral salpingectomy was performed. OUTCOMES: The PSTT patient was cured, which contributed to the symptom relief of TMA. LESSONS: This case report aims to elucidate the relationship between TMA and PSTT, so as to achieve timely diagnosis and treatment and reduce misdiagnosis.

Placental site nodule (PSN): an uncommon diagnosis with a common presentation.

Placental site nodule (PSN) is a rare benign lesion of the intermediate trophoblast which is thought to represent incomplete involution of the placental implantation site. PSN usually presents as menorrhagia, intermenstrual bleeding or an abnormal Pap smear. PSN is benign, but it is important to distinguish it from the other benign and malignant lesions like decidua, placental polyp, exaggerated placental site and placental site trophoblastic tumour. Follow-ups of typical PSNs do not show recurrence or malignant potential. PSN is an uncommon condition which should be suspected in cases of abnormal bleeding, especially following uterine surgical procedures preceding last pregnancy. Timely diagnosis and treatment is necessary to differentiate it from potentially malignant placental lesions with a similar presentation.

Kidney involvement in a patient affected by placental site trophoblastic tumor.

We report a 42-year-old woman who presents a few days after a spontaneous incomplete abortion at the ninth week of pregnancy with hypertension and nephrotic syndrome. Curettage findings and increased values for the ß subunit of human chorionic gonadotrophin were suspicious for a trophoblastic disease. A uterine placental site trophoblastic tumor was diagnosed 2 months later after hysterectomy and treated successfully using chemotherapy. Kidney biopsy showed features consistent with an unusual form of thrombotic microangiopathy characterized by the presence of large thrombus-like structures occluding the capillary lumina and smaller aggregates in the mesangium and along glomerular basement membranes. These deposits were positive for immunoglobulin M, C4, C1q, κ and λ light chains, and fibrinogen. Electron microscopy showed fibrin deposits located primarily in the subendothelial space. The differential diagnosis of this presentation included pre-eclamptic nephropathy, Waldenström disease, lupus anticoagulant glomerulonephritis, systemic lupus erythematosus, and cryoglobulinemic glomerulonephritis. We review the pathogenic mechanisms involved in this case.

Placental site trophoblastic tumor in the ovary of a young child with isosexual precocious puberty.

The authors report a unique case of a primary ovarian placental site trophoblastic tumor (PSTT) in a 30-month-old girl who presented with isosexual precocious puberty of 1 month duration. Laboratory studies revealed mildly elevated beta human chorionic gonadotropin (37.5 mIU/ml; reference range <3.0 mIU/ml) and estradiol (74 pg/ml; reference range 0 to 56 pg/ml) serum levels. A 3.5-cm right ovarian mass was detected radiographically. The tumor was confined to the ovary as proven by the preoperative staging workup and the exploratory laparotomy. Microscopically, it was composed of intermediate trophoblastic cells with angioinvasive growth and deposition of fibrinoid material. The tumor cells were diffusely positive for human leukocyte antigen G, melanoma cell adhesion molecule (CD146), and cytokeratins (AE1/AE3, CK18, and CAM 5.2). Stains for human chorionic gonadotropin, alpha-inhibin, and human placental lactogen showed focal immunoreactivity. Serum markers returned to normal postoperatively. The patient remains disease-free at 24-months follow-up. We propose that tumors morphologically identical to uterine PSTT may rarely occur as gonadal germ cell tumors in children.

Placental site trophoblastic tumor in a patient with secondary infertility and radiological findings consistent with a leiomyoma: a case report.

Placental site trophoblastic tumor (PSTT) is the rarest form of gestational trophoblastic diseases. We report a 40-year-old woman who presented initially after a year of secondary infertility with prolonged menstrual flow. Her last pregnancy ended in a suction dilation and curettage at 8 weeks' gestation for a missed abortion. A hysterosalpingogram revealed a solid 5 x 4.5 cm filling defect impinging on the endometrial cavity suggestive of a submucosal leiomyoma. A vaginal sonogram confirmed the findings. The patient underwent a laparotomy for excision of the 5-cm friable, necrotic mass that was performed by curetting. Pathologic examination of the specimen revealed sheets of intermediate trophoblastic cells characterized by large polyhedral cells and positive human placental lactogen staining consistent with PSTT. The patient eventually underwent total abdominal hysterectomy 4 weeks later with no evidence of residual tumor on histologic examination.

[Luteinized cystic ovarian hyperplasia during a normal pregnancy]. / Hyperplasie kystique lutéinisée des ovaires au cours d'une grossesse normale.

Hyperreactio luteinalis (HRL) is rarely observed in normal pregnancy. Its clinical spectrum consists of benign development of often bilateral lutein cysts and may be revealed by an overproduction of androgens. HRL is more often in relation with an excessive production of human chorionic gonadotropin (hCG) either in trophoblastic disease or in hyperplacentosis (Rh-alloimmunization or diabetes), but in 60% of the cases HRL may occur in normal singleton pregnancies. Many benign or malignant ovarian lesions can mimic HRL during pregnancy. The pathophysiology of HRL in singleton pregnancies involves an increased sensitivity of ovarian stromal cells to hCG. A positive ovarian stimulation test with hCG, recommended three months after delivery may detect a recurrence risk for further pregnancies. Conservative treatment is advised and surgery must be reserved for maternal complications.

Ultrasound diagnosis of uterine arteriovenous fistula associated with placental site trophoblastic tumor.

We report a case of a woman with abnormal vaginal bleeding who had a placental site trophoblastic tumor (PSTT) detected following hysterectomy. Surgery was performed because of a large uterine arteriovenous fistula detected by transvaginal color and pulsed Doppler sonography. Color Doppler sonography revealed a lacunar-type lesion with a marked increase in uterine vascularity, and pulsed Doppler sonography demonstrated a low resistance index. This vascular pattern indicated the formation of blood lacunae and arteriovenous shunts caused by PSTT within the uterine myometrium. This is the first report to describe the ultrasound findings in a case of PSTT complicated by a uterine arteriovenous fistula.

Extrauterine placental site trophoblastic tumour in association with a lithopedion.

AIM: We describe an unusual case of extrauterine placental site trophoblastic tumour located in pouch of Douglas in association with a lithopedion. METHODS AND RESULTS: A 35-year-old female presented with acute abdomen and peritonitis following rectal perforation. The patient gave a history of 5 months amenorrhoea followed by vaginal bleeding 5 years prior to admission. At laparotomy, a lithopedion was found in pouch of Douglas with rectal perforation and peritonitis. The lithopedion was removed, rectal perforation was sutured and a colostomy was performed. The colostomy was closed later and tumour was seen in the colostomy wound as well as attached to the lithopedion removed previously. The patient presented with a repeated episode of rectal perforation and the tumour had spread to colon, small intestine, omentum, mesentery and right ovary. CONCLUSION: A high-grade malignant placental site trophoblastic tumour with aggressive clinical course occurred at an extrauterine site. It complicated calcified abdominal pregnancy and resulted in repeated rectal perforation and peritonitis.

Twenty-five years' clinical experience with placental site trophoblastic tumors.

OBJECTIVE: To describe 34 cases of placental site trophoblastic tumor (PSTT) treated at Charing Cross Hospital over 25 years. STUDY DESIGN: Between 1975 and 2001, 1,685 patients with gestational trophoblastic disease (GTD) were treated; 34 of them had PSTT (2%). The computer database clinical notes and the pathology reports were accessed to obtain data on this patient group. The data were subsequently analyzed using Excel computer software. RESULTS: The mean age of the group was 33 years (95% CI 25-41). The antecedent pregnancy was a full-term, normal one in 18 cases (53%), a molar pregnancy in 7 (21%) and a missed abortion in 5 (15%). The mean interval from the last pregnancy to diagnosis was 3.4 years (95% CI 1.9-4.9). The range of serum hCG concentrations at diagnosis was 0-58,000, 79% with levels < 1,000 and 58% < 500. hCG was raised in all with active disease. The most frequent presenting complaint was vaginal bleeding, in 27 cases (79%). At diagnosis, the disease was localized to the uterus in 15 (44%); there was pelvic involvement in 8 (24%) and lung secondaries in 10 (29%). All seven deaths were disease related (21%); all had lung secondaries and presented more than four years since the last pregnancy. Excluding the seven deaths, the primary treatment was surgery alone in 10 cases (37%) (8 hysterectomies and 2 dilatation and curettages); 4 had surgery followed by adjuvant chemotherapy; 5 had neoadjuvant chemotherapy followed by surgery; 1 had chemotherapy alone, and the disease recurred and was successfully rechallenged; and 5 had surgery between chemotherapy cycles. The most common regimens consisted of EMA/CO and EP/EMA. CONCLUSION: Risk factors for death include lung metastatic involvement (50%) and an antecedent pregnancy interval of four years or more (100%). In contrast, those with no extrapelvic disease or a pregnancy interval of less than four years had 100% survival. In two-thirds of patients with disease limited to the uterus, surgery alone was curative. The WHO scoring system for GTD did not correlate with this outcome. Patients with PSTT should be managed separately from those with other types of GTD, as the disease behavior is different.

Lymphoproliferative disorder of fetal origin presenting as oligohydramnios.

Lymphoma involving the placenta or fetus remains a very rare event. All cases reported to date have shown the lymphoma cells to be of maternal origin in that the tumor cells have preferentially involved the intervillous spaces with sparing of the villi and fetal circulation. We report a novel case of a monoclonal primary placental Epstein-Barr virus (EBV)-associated B-cell lymphoma of fetal origin. The placenta of a 20-week stillborn fetus born to a 19-year-old gravida 1 para 0 woman, presenting with oligohydramnios, showed a large cell infiltrate confined within villi and sparing the intervillous spaces, indicative of preferential involvement of the fetal circulation. Necropsy did not show any other site of involvement by malignant lymphoma or other abnormalities. Immunophenotypic studies showed the tumor cells to be of B-cell phenotype with a relatively high proliferation rate. EBV EBER1 RNA was identified in more than 95% of tumor cells, and polymerase chain reaction studies showed EBV EBNA1 strain type A and wildtype EBV LMP1. Analysis of the immunoglobulin heavy chain by polymerase chain reaction showed a monoclonal B-cell population. In situ hybridization studies using a commercially available probe directed at repeated sequences on the human Y chromosome showed a single intense signal within trophoblastic epithelium and lymphoma cells, indicative of male origin. The mother remains in good health 11 months after delivery.

[Giant placental angioma with polyhydramnios, high level of alpha-fetoprotein and neonatal congenital lactic acidosis]. / Olbrzymi naczyniak lozyska powiklany wielowodziem, wysokim poziomem alfa-fetoproteiny i wrodzona kwasica mleczanowa (congenital lactic acidosis).

Placental tumors are rare in pregnancy. They cause nonspecific complications such as polyhydramnios, fetal anemia, fetal thrombocytopenia and cardiac decompensation with non-immunological hydrops fetalis. In the presented case a very large placental hemangioma was connected with polyhydramnios, premature delivery, fetal anemia and thrombocytopenia, maternal serum alpha fetoprotein elevation and congenital lactic acidosis. After delivery a severe state of the newborn was caused by oligovolemic shock. In the course of the disease the neonatal state steadily deteriorated mainly because of sepsis, cerebral hemorrhage and metabolic acidosis despite adequate therapy. The organic acids assessment in the blood serum of a newborn child showed a very strong signal of lactic acid and an increase in parahydroksyfenylolactic acid. Postmortal examination confirmed prematurity, respiratory distress syndrome, sepsis and cerebral hemorrhage. These symptoms probably resulted from the presence of a placental tumor of considerable size of 12 cm and congenital lactic acidosis, which to our knowledge, have not been described in the available literature until now. In conclusion it should be underlined, that there exists difficult to explain relationship between the presence of a placental haemangioma and severe metabolic changes resulting in high mortality and morbidity of the newborn.

Enfermedad trofoblastica gestacional

La enfermedad trofoblastica gestacional, comprende un amplio espectro de desordenes que van desde la mola hidatiforme benigna al coriocarcinoma maligno y tumor trofoblastico placentario, Recientes avances en patología y citogenética han permitido conocer la etiología de la mola hidatiforme. La Gonodotropina crónica Humana (HCG) es importante en el manejo embarazo molar. La quimioterapia, en cambio, es la piedra angular en el manejo de la enfermedad persistente y recurrente. Nuevos esquemas de quimioterapia han ayudado al manejo de las recurrencias ante el fallo de las drogas usuales.

Perfil clínico-patológico de la mola hidatiforme parcial / Clinical-pathological profile of the hydatiform partial mole

Entre enero de 1979 y diciembre de 1991 se estudiaron 63 mujeres con enfermedad trofoblástica del embarazo. Seis de ellas fueron molas parciales que evolucionaron desfavorablemente: tres como abortos consumados y tres que desarrollaron hipertensión arterial con síndrome nefrótico (una de ellas con tensión arterial de 210/130 mm.Hg. y convulsiones). Del total de molas parciales la mitad fueron estudiadas genéticamente. Dos tuvieron cariotipo diploide y una triploide. Las que cursaron con hCG más elevada fueron tratadas con metotrexito. Ninguna de ellas se embarazaron durante los cinco años subsiguientes. (AU)

Perfil clínico-patológico de la mola hidatiforme parcial / Clinical-pathological profile of the hydatiform partial mole

Entre enero de 1979 y diciembre de 1991 se estudiaron 63 mujeres con enfermedad trofoblástica del embarazo. Seis de ellas fueron molas parciales que evolucionaron desfavorablemente: tres como abortos consumados y tres que desarrollaron hipertensión arterial con síndrome nefrótico (una de ellas con tensión arterial de 210/130 mm.Hg. y convulsiones). Del total de molas parciales la mitad fueron estudiadas genéticamente. Dos tuvieron cariotipo diploide y una triploide. Las que cursaron con hCG más elevada fueron tratadas con metotrexito. Ninguna de ellas se embarazaron durante los cinco años subsiguientes.