Ovarian non-gestational placental site trophoblastic tumor with lung metastasis: further evidence for a distinct category of trophoblastic neoplasm.

We previously described a series of cases which characterize a distinct group of primary ovarian placental site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT) as a non-gestational set consistent with germ cell type/origin. Here we report a new case of ovarian non-gestational PSTT. The patient was a 13 year-old young female admitted for a spontaneous pneumothorax of the left lung. The pathology of lung wedge excision specimen demonstrated metastatic PSTT and ovarian biopsy showed atypical intermediate trophoblastic proliferation which was found to be PSTT in the subsequent salpingo-oophorectomy specimen. In the ovary, the tumor was composed of singly dispersed or small clusters of predominantly mononuclear cells and rare multinucleated cells extensively infiltrating the ovarian parenchyma, tubal mucosa, and paraovarian/paratubal soft tissue. A minor component of mature cystic teratoma (less than 5% of total tumor volume) was present. Immunohistochemically, the neoplastic cells of main tumor were diffusely immunoreactive for hPL, Gata3 and AE1/AE3, and had only rare hCG-positive or p63-positive cells. The morphology and immunohistochemical results support a PSTT. Molecular genotyping revealed an identical genotype pattern between the normal lung tissue and the metastatic PSTT, indicating its non-gestational nature of germ cell type/origin. This case represents the first case of such tumor with distant (lung) metastasis. This case also provides further evidence to support our recommendation that primary ovarian non-gestational intermediate trophoblastic tumors of germ cell type/origin, including PSTT and ETT, should be formally recognized in classification systems.

Placental site trophoblastic tumor (PSTT): a case report and review of the literature.

Placental site trophoblastic tumor (PSTT), also known as atypical choriocarcinoma, syncytioma, chorioepitheliosis or trophoblastic pseudotumor, is a rare gestational trophoblastic disease (0.25-5% of all trophoblastic tumors) and it is composed by neoplastic proliferation of intermediate trophoblasts at placental implantation site. It consists of aggregates or sheets of large, polyhedral to round, predominantly mononucleated cells with a characteristic vascular and myometrial invasion. Main differential diagnoses are gestational choriocarcinoma (GC) and epitelioid trophoblastic tumor (ETT). We present a case of PSTT in a 25-year-old woman. Neoplastic cells showed moderate/high nuclear pleomorphism, abundant amphophilic, eosinophilic and clear cytoplasm, numerous mitotic figures (10 mitoses/10 HPF), and myometrial invasion. Other features are necrosis, vascular invasion with replacement of myometrial vessels by tumor cells and hemorrhage. The patient showed typical low serum ß-hCG levels and high serum humane placental lactogen (hPL) levels.

Retrospective analysis of clinical features and fertility outcomes with fertility-sparing treatment of placental site trophoblastic tumor.

OBJECTIVE: To analyze the methods, feasibility, efficiency, and fertility outcomes of fertility-sparing treatment for patients with placental site trophoblastic tumor (PSTT). METHODS: Clinical data of patients diagnosed with PSTT between April 1998 and April 2020 from Peking Union Medical College Hospital (PUMCH) were retrospectively collected. The clinical features, treatment, and outcomes of patients received fertility-sparing treatment were analyzed and compared with patients suffered hysterectomy. RESULTS: In total, 126 patients were included in the study and 29 of them received fertility-sparing treatment. Besides significantly younger age and lower proportion of antecedent term delivery were seen in fertility-sparing group than hysterectomy group, no significant differences were observed in stage, serum ß-hCG level, or interval from antecedent pregnancy between the two groups. Conservative surgery was selected individualized and none of them suffered salvage hysterectomy. Patients with clinical or pathological high-risk factors received adjuvant chemotherapy, yet the fertility-sparing treatment did not significantly lengthen chemotherapy duration. All patients in fertility-sparing group achieved complete remission without relapse after 36 to 176 months of follow-up and had sixteen healthy term delivery more than one year after the treatment. CONCLUSIONS: Fertility-sparing treatment for PSTT can be considered for young patients with localized uterine lesions who strongly desire to preserve their fertility potential. With individualized conservative surgery and selected adjuvant chemotherapy, fertility-sparing treatment will not influence the risk of relapse or overall survival and patients will achieve favorable pregnancy and live birth outcomes.

Placental Site Trophoblastic Tumor in Mediastinal Recurrence of Mixed Germ Cell Tumor of the Testis: Report of a Case and Review of the Literature.

We present a rare case of a 42-year-old man diagnosed with a placental site trophoblastic tumor in combination with teratoma in a mediastinal recurrence of a testicular germ cell tumor post-orchiectomy and chemotherapy. To the best of our knowledge, this is the eighth case of placental site trophoblastic tumor in a male reported so far in the English literature. The purpose of this case report is to add data to the existing literature, review the literature, discuss the differential diagnoses with emphasis on morphologic and immunohistochemical differences between trophoblastic tumors, and highlight the management implications of a correct diagnosis.

A Rare Case of Combined Choriocarcinoma and Placental Site Trophoblastic Tumor Presenting as Skin Lesion: A Case Report.

BACKGROUND Despite the tendency to metastasize widely, Gestational Trophoblastic Neoplasia (GTN) is one of the most curable solid tumors with chemotherapy. CASE REPORT A 41-year-old female, G4P2A2, presented with a slowly growing lump on the left side of the scalp associated with a headache. The patient had intermittent, sharp left eye pain which radiated to the side of her face, photophobia, early morning blurring of vision, and nausea. Palpation over scalp lesion produced deep retro-orbital pain and pain was exacerbated with bending over. An ophthalmological evaluation was unremarkable. Ultrasonography (USG) of the left scalp showed an intramuscular mass superficial to the left frontal bone. During excision biopsy, the mass was found to be invading the frontal bone. Histopathology showed a metastatic trophoblastic tumor with mixed features of choriocarcinoma and placental site trophoblastic tumor. A pregnancy test was positive, the beta HCG level was elevated but USG did not show intrauterine pregnancy. CT head demonstrated an intracranial, dural-based mass that extended against the brain but did not breach the pial membrane. CT chest, abdomen, pelvis, and PET scan showed no evidence of metastatic disease. She was successfully treated with resection of the transcranial lesion followed by aggressive chemotherapy - Etoposide, Methotrexate, Actinomycin-D, Vincristine, and Cyclophosphamide. CONCLUSIONS This was an unusual case of GTN due to its primary presentation as skin metastasis, without any lung metastasis and no identifiable primary lesion. It is also very unusual to see a combination of choriocarcinoma and placental site trophoblastic tumor cells in the same tumor mass.

Outcomes and prognostic factors of placental-site trophoblastic tumor: a retrospective study of 58 cases.

PURPOSE: Our goal was to assess the outcomes and explore the prognostic factors for patients with placental-site trophoblastic tumor (PSTT) through this retrospective analysis. METHODS: 2043 patients with gestational trophoblastic neoplasia (GTN) were registered at two tertiary hospitals between January 2003 and March 2021, of whom 58 (2.8%) were diagnosed with PSTT. We retrospectively analyzed the clinico-pathological characteristics, treatments, outcomes and prognostic factors. RESULTS: Only 4 patients died and 5 patients experienced a recurrence. Patients (n = 49) with stage I disease had a favorable prognosis, surgery with (n = 21) or without (n = 28) chemotherapy made no significant difference in overall survival (OS) (p = 0.251) or disease-free survival (DFS) (p = 0.425). 3 patients with stage I had fertility preserving surgery and successful pregnancy was achieved in 2 of them. The outcome of patients with advanced disease was poor. Univariate analysis revealed serum ß-hCG levels at diagnosis, FIGO stage IV and metastatic disease were significant predictors of both overall survival and disease-free survival. However, multivariate analysis indicated stage IV was the only significant independent predictor of adverse OS, while metastatic disease was the only significant independent predictor of adverse DFS. CONCLUSION: Surgery alone is sufficient for patients with stage I disease without high-risk factors. The prognosis of patients with advanced stage disease remains poor. Stage IV and metastatic disease were the most critical risk factors.

The role of the ultrasound examination in atypical placental site trophoblastic tumour differential diagnosis and management. A case report.

Placental site trophoblastic tumour (PSTT) is a very rare and unique form of gestational trophoblastic tumour, representing about 1-2% of all gestational trophoblastic tumours. Usually, the pattern is a slow growing nodule implicating the endometrium and myometrium, accompanied by abnormal uterine bleeding. Three ultrasound types of PSTT are described, but thereis no specific characteristic for diagnosis. We present the case of a patient with an atypical placental site trophoblastic tumour diagnosed two months after a caesarean scar pregnancy. In the presented case there are several particularities, such as the rapid growth and progression of the tumour, the limitation to the myometrium and the difficulty of the differential diagnosis and approach.

"It's not lupus". A placental site trophoblastic tumor presenting as a lupus-like paraneoplastic syndrome. A grand round case.

Introduction: Placental site trophoblastic tumor (PSTT) is a rare subtype of gestational trophoblastic disease. Association of PSTT and nephrotic syndrome is exceedingly rare and has been described in 8 cases thus far. In all cases hysterectomy was performed within months after onset of symptoms, leading to immediate remission of nephrotic syndrome, except for one patient who died of complications of PSTT. Case: We describe the history of a woman in which PSTT was discovered years after onset of nephrotic syndrome. Kidney biopsy revealed lupus-like mesangiocapillary nephritis and over time the patient developed additional symptoms mimicking systemic lupus erythematosus (SLE). Discussion: We provide an overview of the literature on this clinical entity and elaborate on its pathophysiology. In addition, we reflect on the phenomenon of anchoring bias, that led physicians to assume the patient had SLE without questioning this diagnosis in the light of the unexplained finding of increased tumor markers.

Placental site trophoblastic tumour and epithelioid trophoblastic tumour.

Placental site trophoblastic tumour (PSTT) and epithelioid trophoblastic tumour (ETT) are the rarest subtypes of gestational trophoblastic disease (GTD). Their diagnosis is complicated and lacks specific and sensitive tumour markers. They are slow-growing tumours and can occur months to years after any type of antecedent pregnancy. The primary treatment for localised disease is hysterectomy. However, extra-uterine invasion and/or metastasis occur in about one-third of cases and still cause death in a small number. Most patients are young; hence, fertility preservation is a consideration. The major obstacle for prognosis is chemotherapy resistance. The current understanding of these tumours remains elusive and no randomized controlled trials have been done. Even those centres treating a large number of patients with GTD will infrequently manage PSTT/ETT. In this review, we assess progress in the understanding of the disease and discuss four main clinical challenges - establishing conformity of practice, devising a risk-adapted approach to clinical management, establishing long-term follow-up data and evaluating therapies for poor prognosis and multi drug-resistant patients.

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.

Management of placental site trophoblastic tumor: Two case reports.

RATIONALE: Placental site trophoblastic tumor (PSTT) is a very rare malignant tumor, belonging to a family of pregnancy-related illnesses, called gestational trophoblastic diseases (GTD). Less than 300 cases of PSTT have been reported in literature, with an incidence of ≈ 1/50,000-100,000 pregnancies representing only 0.23% to 3.00% of all GTDs. PATIENT CONCERNS: Our report describes 2 additional cases of PSTT outlining their main diagnostic features and the subsequent management. The first case presented contemporary to a persistent hydatidiform mole in a 37-year-old woman, para 2042; whereas the second one originated 5 years after a miscarriage in 43-year-old woman, para 1031 with a previous diagnosis of breast cancer, and shared some features with placental site nodule (PSN), a benign condition. DIAGNOSIS: The first case had a difficult diagnosis because there was an amenorrhea of 11th week with high serum beta-human chorionic gonadotropin (beta-HCG) and an initial ultrasound image of vesicular mole. After the Dilatation and Curettage, histology confirmed the previous hypothesis. However, the final histology of PSTT was obtained after major surgery. On the contrary, the diagnosis of the second case was less challenging but surprising, thanks to a routine trans-vaginal ultrasound showing a suspicious endometrial thickness positive for PSTT at a subsequent hysteroscopic guided biopsy. INTERVENTIONS: The treatment consisted of hysterectomy and subsequent follow up. Lymphadenectomy or lymph node sampling were not performed due to the initial stage of the disease. OUTCOMES: In the first case, there were high values of serum beta-HCG that plummeted after the surgery, whereas in the second one they had been always negative. Hereafter, both went through a follow up with periodic serum oncological markers, imaging studies and clinical evaluation, which have showed negative result for 3 years and 15 months, respectively. LESSONS: A detailed gynecological ultrasound examination could be extremely helpful to understand the next diagnostic step of echo-guided D&C or hysteroscopic biopsy and for a pre-operative staging assessment. On the contrary, determining the serum beta-HCG's curve is crucial just in case of an initial positive value to pursue clinical evaluation and follow-up. In case of good prognostic factors, the main therapy remains hysterectomy.

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 trophoblastic tumor in early stage: a case report.

Here, the authors present a case of a placental site trophoblastic tumor (PSTT) in a 28-year-old gravida 1 para 1 living 1 woman three months after vaginal delivery of a female infant at diagnosis in 2014. The patient was FIGO Stage I and finally underwent a total laparoscopic hysterectomy with ovarian conservation. Subsequently, the patient received two cycles of EMA/CO chemotherapy. Patient is on regular follow-up (clinical exam, ß-hCG tests, pelvic and abdominal sonography) and has shown no signs of local or systemic recurrence for 24 months.

Diagnosis and management of a rare case with placental site trophoblastic tumor.

OBJECTIVE: Placental site trophoblastic tumor (PSTT) is rare and is characterized by a slow growth. The objective of this report is to present a case of PSTT associated with irregular vaginal spotting that occurred 1 year after normal vaginal delivery. CASE REPORT: This report provides interesting ultrasound, hysteroscopy, and histology findings of PSTT. It is difficult to make a clinical diagnosis of PSTT at an early stage. Without the use of immunohistochemical analysis, PSTT may evade histological detection. An operative hysteroscopy using electrocauterization reduces active bleeding during the removal of PSTT with markedly engorged tumor vessels. CONCLUSION: Transvaginal sonography using color Doppler imaging plays a vital role in identifying residual PSTT with microscopic infiltration to the myometrium and a negative serum ß-human chorionic gonadotropin level.

[Placental site trophoblastic tumor with atypical choriocarcinoma. Case Report]. / Tumor trofoblástico del sitio placentario con evolución a coriocarcinoma. Caso clínico.

BACKGROUND: The placental site trophoblastic tumor is the second rarest tumor of trophoblastic disease, with less than 300 cases reported. Clinical presentation it's a diagnostic challenge, it has an unpredictable malignant potential, and it can develop choriocarcinoma and even have simultaneous presentation. CASE REPORT: We present the case of a 34 year old woman with chronic transvaginal bleeding since her last cesarean (five months ago), with histologic diagnosis of placental site trophoblastic tumor and inmuno- histoquimic report of choriocarcinoma, this early diagnosis improves her survival prognostic. CONCLUSION: placental site trophoblastic tumor and their evolution to choriocarcinoma have a low rate but it's possible. That it's the reason why complete diagnosis with biopsy, histologic and inmunohistoquimic report is mandatory to improve therapeutic.

Placental Site Trophoblastic Tumor: A Distinct Entity of Gestational Trophoblastic Disease Experience from a Tertiary Referral Center in Hong Kong.

OBJECTIVE: To review the clinical and pathological characteristics of patients with placental site trophoblastic tumor (PS TT) managed in a tertiary referral center in Hong Kong. STUDY DESIGN: Patients with a diagnosis of PSTT from 1995 to 2012 were identified from a computer database. Clinical and patho- logical data were obtained from medical records and the electronic database. RESULTS: Ten patients with PSTT were identified. Only 4 patients (40%) had disease confined to the uterus at presentation (Stage I). The most common site of metastasis was the lung. Four patients had pretreatment serum hCG levels <1,000 IU/L, and all of them had disease 'confined to the uterus. Of the 4 patients with Stage I disease 3 had hysterectomy only and 1 had both hysterectomy and chemotherapy. All 4 patients achieved complete remission; although 1 of them had a recurrence successfully treated with che- motherapy. For patients with Stage III/IV disease most of them had both hysterectomy and chemotherapy. Only 1 patient (20%) was alive without evidence of disease. CONCLUSION: Patients with Stage I disease have excellent prognosis after hysterectomy, and adjuvant treatment is not recommended. A low pretreatment serum hCG level (<1,000 IU/L) was a good predictor of early stage disease. The prognosis for patients with metastatic disease was poor despite surgery and com- bination chemotherapy.

Placental site trophoblastic tumor: a case report and review of the literature.

We present a case of a gravida 1 para 1 woman, who presented with an 11-month history of amenorrhea after cesarean delivery. The patient was taking birth control pills at the time of presentation. She was observed with a slight elevation of serum ß-hCG level, an enlarged heterogeneous uterus and hematometra. A biopsy was performed, and the patient was diagnosed with placental site trophoblastic tumor; the patient then underwent surgery. Placental site trophoblastic tumor is the rarest form of gestational trophoblastic disease, derived from intermediate trophoblast cells. It does not have a pathognomonic appearance; therefore, correlation with medical history, as well as results of laboratory tests and pathological analysis is mandatory. It is a relatively chemoresistant tumor, posing considerable therapeutic challenges; patients with localized disease are managed with surgery and those with metastatic disease require additional chemotherapy. Herein, we review the main features of this entity and top differential diagnosis, as the rarity of this tumor is associated with imaging and pathological pitfalls, reinforcing the need for further experience in this field.

Placental site trophoblastic tumor and choriocarcinoma: an unusual presentation.

BACKGROUND: Mixed trophoblastic tumor composed of choriocarcinoma and placental site trophoblastic tumor was diagnosed on histopathology slides review, is a very rare mixed tumor and cured after adjuvant chemotherapy. CASE PRESENTATION: Twenty-nine years old, Para 2 female presented with 4 months history of irregular vaginal bleeding after an uncomplicated vaginal delivery. Abdominal examination showed 14 weeks uterus with ß-hCG level of 14,889 mIU/ml. Slides review confirmed the diagnosis of placental site trophoblastic tumor (PSTT). Patient underwent total abdominal hysterectomy and resection of anterior vaginal wall mass. Post operative (48 h) ß-hCG level was 6016 mIU/ml. Final pathology showed mixed trophoblastic tumor composed of choriocarcinoma (CC) and PSTT. Adjuvant chemotherapy started and continued for three cycles after achieving normal ß-hCG. CONCLUSION: PSTT is a rare disease and potentially curable. Differential diagnosis in women presented with vaginal bleeding and a uterine mass in the post partum period must include gestational trophoblastic disease.