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Acta Bio-medica : Atenei Parmensis Dec 2019Yolk sac tumor (YST) is a rare tumor that usually occurs in the first two decades of life. It is considered the second most common malignant germ cell tumor of the...
Yolk sac tumor (YST) is a rare tumor that usually occurs in the first two decades of life. It is considered the second most common malignant germ cell tumor of the ovary, characterized by a rapid growth and a bad prognosis due to the frequent metastasis. We report the case of a 12-year-old girl who came to our observation for an acute abdominal pain. Clinical examination evidenced a vague mass in the suprapubic region and a lower abdomen tenderness, the US imaging revealed a complex lesion of the left ovary (19 x 13 cm) and the alpha-fetoprotein (AFP) resulted high (5858 ng/mL). Computed tomography (CT) revealed a large pelvic mass. The treatment consisted of debulking surgery of yolk sac tumor followed by 4 cycles of BEP protocol (Bleomycin, Etoposide, Cisplatin). After 3 years of follow-up there was no evidence of disease recurrence. (www.actabiomedica.it).
Topics: Abdomen, Acute; Adolescent; Endodermal Sinus Tumor; Female; Humans; Ovarian Neoplasms
PubMed: 31910193
DOI: 10.23750/abm.v90i4.9017 -
Beijing Da Xue Xue Bao. Yi Xue Ban =... Dec 2019Primary mediastinal yolk sac tumor, which is also known as endodermal sinus tumor, is a rare but lethal neoplasm and its prognosis is very dismal. The current treatment...
OBJECTIVE
Primary mediastinal yolk sac tumor, which is also known as endodermal sinus tumor, is a rare but lethal neoplasm and its prognosis is very dismal. The current treatment for this tumor is controversial, and chemotherapy combined with resection of residual lesions is adopted sometimes. We summarized the experience of seven primary mediastinal yolk sac tumors treated with platinum-based chemotherapy and extended resection in Peking University First Hospital.
METHODS
Clinicopathological data of the patients with primary mediastinal yolk sac tumor who received operation in Peking University First Hospital between August 2014 and August 2018 were collected and analyzed retrospectively.
RESULTS
We experienced seven primary mediastinal yolk sac tumors during this period. Computed tomography scan revealed an anterior mediastinal tumor in all the patients and all of them had markedly raised alphafetoprotein (AFP) and normal β-human chorion gonadotropin (β-HCG). Five patients underwent needle core biopsy before treatment, which showed a mediastinal yolk sac tumor. All of these patients received preoperative platinum-based chemotherapy and they all presented partial response according to computed tomography. Two other patients did not receive preoperative biopsy, so they directly underwent extended resection. All of the seven patients underwent operation successfully and two of them experienced postoperative complications, including one with pneumonia and the other with atelectasis. R0 resection was achieved in six patients and R1 resection was achieved in the other patient. According to postoperative pathology, there were one microcyst subtype, one adenoid subtye, one giant capsule subtype and two hybrid subtypes. Surprisingly, there were no yolk sac tumor tissue in the other two patients after preoperative chemotherapy. All the patients received postoperative chemotherapy, excluded one patient who was unable to tolerate chemotherapy after operation. Three patients experienced postoperative pulmonary metastases within one year and two of them died soon. The other patient received chemotherapy and immunotherapy after recurrence and he was alive at the time of writing. Four other patients were alive without recurrence and metastasis.
CONCLUSION
Primary mediastinal yolk sac tumor is rare and its prognosis is poor. A multimodality approach including adjuvant chemotherapy and resection of residual lesions is the optimal treatment and it may lead to long-term survival.
Topics: Endodermal Sinus Tumor; Humans; Male; Mediastinal Neoplasms; Mediastinum; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 31848510
DOI: 10.19723/j.issn.1671-167X.2019.06.020 -
Taiwanese Journal of Obstetrics &... Nov 2019To report a rare case of endometrial yolk sac tumor (YST) and review published cases of YST of the endometrium. (Review)
Review
OBJECTIVE
To report a rare case of endometrial yolk sac tumor (YST) and review published cases of YST of the endometrium.
CASE REPORT
A 68-year-old female presented with intermittent vaginal spotting for nine months. An endometrial biopsy showed adenocarcinoma. Complete surgical staging operation was performed and the final pathology revealed stage II endometrial yolk sac tumor. The post-operative α-fetoprotein (AFP) level was 133.4 ng/mL. Post-operative adjuvant chemotherapy with bleomycin, etoposide, and cisplatin (BEP) regimen was prescribed for 6 cycles. AFP levels were normal before the fourth cycle of chemotherapy. She is disease free 6 months after completion of therapy.
CONCLUSION
Primary YSTs arising in the endometrium is an extremely rare disease especially in postmenopausal women. Complete surgical staging operation with adjuvant chemotherapy will lead to good outcome in this disease.
Topics: Aged; Antineoplastic Agents; Biopsy; Chemotherapy, Adjuvant; Endodermal Sinus Tumor; Endometrial Neoplasms; Endosonography; Female; Humans; Hysterectomy; Laparotomy; Vagina
PubMed: 31759539
DOI: 10.1016/j.tjog.2019.09.020 -
Current Oncology (Toronto, Ont.) Oct 2019Prostatic yolk sac tumour is a germ cell tumour with a wide range of age of occurrence, unusual anatomic locations, diverse morphologic patterns, and aggressive biologic...
Prostatic yolk sac tumour is a germ cell tumour with a wide range of age of occurrence, unusual anatomic locations, diverse morphologic patterns, and aggressive biologic behavior, posing challenges both to diagnosis and clinical management. We report a rare case of primary yolk sac tumour of the prostate with extensive local and liver metastasis, the latter of which exhibited sheets of small blue cells expressing CD99 and focal sall4 on biopsy. Positivity for CD99 and gata3 in the initial biopsy raised the differential diagnosis of Ewing sarcoma and poorly differentiated carcinoma. The primary tumour demonstrated an admixture of solid and glandular growth patterns and occasional Schiller-Duval bodies. A panel of immunohistochemical stains showing positivity for AE1/3, sall4, cdx2, and focal alpha-fetoprotein, and negativity for oct-4, facilitated the diagnosis. A thorough review of the literature and our current report indicate that a large tumour load, incomplete tumour resection, limited response to preoperative neoadjuvant chemotherapy, and late stage of the disease are predictive factors for a poor clinical outcome.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Endodermal Sinus Tumor; Fatal Outcome; Humans; Liver Neoplasms; Male; Neoadjuvant Therapy; Neoplasms, Germ Cell and Embryonal; Prostatic Neoplasms; Stem Cell Transplantation; Tomography, X-Ray Computed; alpha-Fetoproteins
PubMed: 31708666
DOI: 10.3747/co.26.5179 -
Medicine Oct 2019Primary thoracic dumb-bell yolk sac tumor (YST) with both epidural and extraspinal extension is a rare disease with no standard curative managements yet. The objective...
RATIONALE
Primary thoracic dumb-bell yolk sac tumor (YST) with both epidural and extraspinal extension is a rare disease with no standard curative managements yet. The objective of this study is to report a primary thoracic dumb-bell YST presenting with severe spinal cord compression successfully treated with posterior-only approach operation, followed by chemotherapy. The management of these unique cases has not been fully documented.
PATIENT CONCERNS
A 26-mounth-old, previously healthy girl presented with progressive numbness and weakness of the lower extremities. Neurological examination revealed paralysis of both lower extremities, sensory disturbance below T-8 and bladder-bowel dysfunction.
DIAGNOSIS
CT and MRI of spine showed a dumb-bell mass lesion with both epidural and extraspinal extension through enlarged intervertebral foramina and marked spinal cord compression at T7-T9. The AFP level was 13738 ng/ml. Preoperative puncture and Postoperative pathology confirmed the diagnosis of YST.
INTERVENTIONS
By needle biopsy, we identified the pathological diagnosis is YST. Subsequently, the patient was treated with one-stage posterior-only approach operation, followed by 9 courses of chemotherapy based on cisplatin, bleomycin, etoposide.
OUTCOMES
The patient has a complete neurologic recovery and remains recurrence free as of more than 2 years after the completion of operation. There were no other complications associated with the operation during the follow-up period.
LESSONS
YST should be considered in the range of children with thoracic dumb-bell tumor presenting with spinal cord compression. Needle biopsy is valuable for preoperative diagnosis and design of the treatment strategy. If there is no evidence of CSF spread, metastasis or multiple diseases, it is effective to remove tumors as thoroughly as possible immediately, avoid further nerve injury and conduct enough chemotherapy. This case suggests that this treatment strategy is an effective option for primary YST with both epidural and extraspinal extension and severe spinal cord compression.
Topics: Antineoplastic Combined Chemotherapy Protocols; Child, Preschool; Decompression, Surgical; Endodermal Sinus Tumor; Female; Humans; Laminectomy; Spinal Cord Compression; Spinal Neoplasms; Thoracic Vertebrae; Treatment Outcome
PubMed: 31651872
DOI: 10.1097/MD.0000000000017610 -
Journal of Investigational Allergology... Aug 2019
Topics: Chemotherapy-Induced Febrile Neutropenia; Child, Preschool; Desensitization, Immunologic; Endodermal Sinus Tumor; Female; Filgrastim; Hematologic Agents; Humans; Treatment Outcome; Vaginal Neoplasms
PubMed: 31478530
DOI: 10.18176/jiaci.0399 -
Head and Neck Pathology Jun 2020The classification of sinonasal adenocarcinoma (SNAC) is complex. The high-grade, non-intestinal SNAC group is particularly heterogeneous, with tumors showing widely...
SMARCB1 (INI-1)-Deficient Adenocarcinoma of the Sinonasal Tract: A Potentially Under-Recognized form of Sinonasal Adenocarcinoma with Occasional Yolk Sac Tumor-Like Features.
The classification of sinonasal adenocarcinoma (SNAC) is complex. The high-grade, non-intestinal SNAC group is particularly heterogeneous, with tumors showing widely variable morphology. SMARCB1 (INI-1)-deficient sinonasal carcinoma is a newly described, aggressive tumor that usually resembles sinonasal undifferentiated carcinoma (SNUC) or non-keratinizing squamous cell carcinoma; however, glandular differentiation has been rarely reported and this feature may be under-recognized. We present a dedicated series of 12 SMARCB1-deficient SNACs. All tumors had an oncocytoid/plasmacytoid cytomorphology with variable degrees of glandular differentiation consisting of tubules and cribriform structures with foci of intracellular or intraluminal mucin. Three of 12 tumors exhibited foci of yolk sac tumor-like histologic features. The tumors were uniformly high-grade, with nuclear pleomorphism, elevated mitotic rates and frequent necrosis. By immunohistochemistry, all tumors were entirely SMARCB1-deficient, and 10 of 12 were CK7-positive. Occasional expression of CDX2 (4 of 12), CK20 (3 of 12), and p40 (3 of 10) was seen. Expression of yolk sac markers was variably present in tumors that harbored yolk sac-like areas but also tumors that did not: glypican-3 (10 of 11), SALL4 (6 of 11), HepPar-1 (4 of 11), PLAP (1 of 10), and AFP (1 of 11). SMARCB1-deficient sinonasal carcinoma, particularly the oncocytoid/plasmacytoid form, can demonstrate variable degrees of glandular differentiation. This unexpected morphology combined with variable immunohistochemical results may lead to misdiagnoses of high-grade intestinal or non-intestinal SNAC, myoepithelial carcinoma, or even yolk sac tumor or metastatic hepatocellular carcinoma.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Endodermal Sinus Tumor; Female; Humans; Male; Middle Aged; Paranasal Sinus Neoplasms; Paranasal Sinuses; SMARCB1 Protein; Young Adult
PubMed: 31468350
DOI: 10.1007/s12105-019-01065-7 -
Modern Pathology : An Official Journal... Dec 2019Uterine yolk sac tumors have gained increased recognition in recent years. The current study is a multi-faceted examination of yolk sac tumor-like phenotypes in...
Uterine yolk sac tumors have gained increased recognition in recent years. The current study is a multi-faceted examination of yolk sac tumor-like phenotypes in endometrial tumors, based on an analysis of 3 groups of uterine tumors: Group 1: 9 endometrial tumors that had been classified as yolk sac tumor, or as having a yolk sac tumor component, were assessed with a 35-marker immunohistochemical panel, with the goal of defining their immunophenotypic spectrum; Group 2, comprised of 70 endometrial carcinomas of various histotypes, were analyzed for their expression of SALL4, Glypican-3, and AFP, to assess the specificity of these markers for yolk sac tumors relative to endometrial carcinomas; Group 3, comprised of 626 archived cases of endometrial carcinoma/carcinosarcoma, reviewed to define the frequency of yolk sac tumor-like morphology therein. Yolk sac tumor areas in the Group 1 cases were consistently immunoreactive for SALL4 and Glypican-3; variably positive for AFP (89%), Villin (89%), PLAP (78%), 34βE12 (67%), CAM 5.2 (62.5%), EMA (56%), CD117 (50%), p16 (50%), CDX2 (44%), p53 (44% aberrant), MOC31 (37.5%), CK7 (33%), GATA3 (33%), CK5 (25%), and PAX8 (11%); and were negative for CD30, Napsin A, OCT4, estrogen, androgen, and progesterone receptors. 29 (41%) of the 70 group-2 cases expressed at least one of the 3 markers, and 96% of the positive cases was a high-grade histotype. Glypican-3, SALL4, and AFP were positive in 30, 20, and 2.8% of group-2 cases respectively; however, co-expression of any 2, or all 3 markers was uncommon (<9 and 1.4% of cases respectively). Potential yolk sac tumor-like morphology was identified in 5 (0.8%) of 626 group-3 cases, and three were ultimately deemed to be true yolk sac tumor phenotypes based on their morphologic and immunophenotypic similarity to the group 1 cases. These findings highlight the broad immunophenotypic spectrum of uterine yolk sac tumors, the potential pitfalls associated with using immunophenotypes alone to define yolk sac tumor differentiation in endometrial carcinoma, and the utility and limitations of morphologic assessment to identify yolk sac tumors at this site.
Topics: Biomarkers, Tumor; Endodermal Sinus Tumor; Endometrial Neoplasms; Female; Humans; Immunohistochemistry
PubMed: 31375771
DOI: 10.1038/s41379-019-0341-6 -
BMJ Case Reports Jul 2019Yolk sac tumour (YST), the most common germ cell tumour (GCT) in infants and children, accounts for 80% of GCTs in this age group. It is observed in only 2.4% of adult...
Yolk sac tumour (YST), the most common germ cell tumour (GCT) in infants and children, accounts for 80% of GCTs in this age group. It is observed in only 2.4% of adult patients in their pure form since up to 42% of mixed GCTs have some component of the yolk sac. We present a 46-year-old cryptorchid man who presented with complaints of abdominal lump, pain and altered bowel habits since 1 month. CT scan of the abdomen revealed a mass arising from the pelvic cavity with feeders predominantly from the left gonadal vessel. Mass was excised via exploratory laparotomy and sent for histological examination. Microscopic examination was suggestive of pure YST. Immunohistochemistry stained positive for cytokeratin, placental-like alkaline phosphatase, Glypican 3 and alpha fetoprotein. The patient succumbed prior to commencing chemotherapy. Pure YSTs of post-pubertal origins are extremely rare and are more aggressive than their prepubertal counterparts.
Topics: Cryptorchidism; Endodermal Sinus Tumor; Humans; Male; Middle Aged; Testicular Neoplasms; Tomography, X-Ray Computed
PubMed: 31337626
DOI: 10.1136/bcr-2019-229541 -
Cancer Cytopathology Aug 2019Mesonephric adenocarcinomas are rare neoplasms which most commonly arise in the lateral cervix and vagina. Tumors with similar morphologic, immunophenotypic, and...
BACKGROUND
Mesonephric adenocarcinomas are rare neoplasms which most commonly arise in the lateral cervix and vagina. Tumors with similar morphologic, immunophenotypic, and molecular characteristics recently have been described in the uterine corpus and ovary. Herein, the authors sought to characterize the cytomorphologic features of adenocarcinomas exhibiting mesonephric-like differentiation arising in the upper gynecologic tract.
METHODS
Institutional databases were queried retrospectively for tumors of the upper gynecologic tract described as a "tumor of Wolffian origin" or "with mesonephric features" between 2007 and 2017. All available cytologic material was reviewed. Cytomorphologic characteristics were evaluated by 3 pathologists.
RESULTS
The current study cohort consisted of 8 cases taken from 7 patients. Primary sites included the ovary (3 cases); endometrium (4 cases); and pelvis, not otherwise specified (1 case). All cases demonstrated tight 3-dimensional clusters of overlapping cells. Additional architectural features included tubular (5 of 8 cases; 63%) and papillary (3 of 8 cases; 38%) formations. Cells were small with scant (7 of 8 cases; 88%) to moderate (1 of 8 cases; 12%) cytoplasm. Three of the 8 cases (38%) demonstrated extracellular hyaline globules. Nuclei were uniform in size (6 of 8 cases; 75%) or showed mild anisonucleosis (2 of 8 cases; 25%). Nuclear grooves and indentations were observed in all cases. Mitoses (5 of 8 cases; 63%) and apoptotic bodies (4 of 8 cases; 50%), when present, were rare. No necrosis was noted.
CONCLUSIONS
Adenocarcinomas exhibiting mesonephric-like differentiation show a monotonous population of small cells with scant to moderate cytoplasm and abundant nuclear grooves arranged in tight, overlapping, 3-dimensional clusters. Occasionally, papillary or tubular architecture, as well as extracellular hyaline globules, may be seen. These features should prompt further testing (eg, immunohistochemistry) to confirm the diagnosis and to exclude potential mimics.
Topics: Adenocarcinoma; Adult; Aged; Endometrial Neoplasms; Endometrium; Female; Humans; Mesonephroma; Middle Aged; Ovarian Neoplasms; Ovary; Retrospective Studies
PubMed: 31318491
DOI: 10.1002/cncy.22160