Authors: Deyin Xing, Sharon X Liang, Faye F Gao...

Given the association of mesonephric adenocarcinoma (MA) of the uterine cervix with florid mesonephric hyperplasia, one would expect MAs to rarely arise in other anatomical locations that harbor mesonephric remnants. In contrast, mesonephric-like adenocarcinoma (MLA) is thought to arise from Müllerian origin without an association with mesonephric remnants. The current case series characterizes 4 cases of MA arising in the urinary bladder (1 woman and 3 men), 1 case of MA in the perirenal region (woman), and 1 case of MLA in the ureter (woman). All cases displayed morphologic features similar to MA of the uterine cervix and MLA of the ovary and endometrium, characterized by predominant tubular and focal glandular/ductal architecture. Mesonephric remnants in the bladder wall were closely associated with adjacent MA in cases 1 and 4. MLA in case 6 was associated with mesonephric-like proliferations and endometriosis. All cases (6/6) were diffusely positive for Pax8, and all displayed a luminal pattern of CD10 staining, except case 4 for which CD10 immunostain was not available for review. Gata3 was either focally positive (cases 1, 2, and 6), negative (case 3), or diffusely positive (case 5). TTF-1 was diffusely expressed in cases 1 and 3 and negative in cases 2, 5, and 6. Although a KRAS G12C somatic mutation was detected in case 6, hotspot mutations in KRAS, NRAS, and PIK3CA were not present in other tested cases. Our study demonstrates that MAs and MLAs of the urinary tract share similar histopathogenesis, morphology, and immunophenotype to their counterparts in the female genital tract. We propose that, in the urinary tract, MA might be classified as a distinctive tumor that arises from mesonephric remnants or presumed Wolffian origin if they are not related to Müllerian-type precursors. The tumor displaying similar morphology and immunoprofile to MA but associated with Müllerian-type precursors should be classified as MLA.

Topics: Male; Female; Humans; Uterine Cervical Neoplasms; Proto-Oncogene Proteins p21(ras); Adenocarcinoma; Mesonephroma; Urinary Tract

PubMed: 36788068
DOI: 10.1016/j.modpat.2022.100031

Authors: Teresa Iscar, Carolina Arean, Luis Chiva...

Topics: Adolescent; Endodermal Sinus Tumor; Female; Humans; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms

PubMed: 33931463
DOI: 10.1136/ijgc-2021-002538

Authors: F Carvalho, B Leitão, A Reis...

Prepubertal testis tumours (T.T) are rare, with different characteristics and clinical course to adults and with a better prognosis. The authors report the experience of Maria Pia Hospital's over a 15 year period. There were 8 cases of T.T.: 3 Yolk sac tumours, 3 teratomas, 1 Leydig cell tumour and 1 epidermoide cyst. All patients are submitted to an inguinal orchiectomy. Follow-up ranged between six months and 14 years. There was no recurrence of the disease in seven patients are without disease recurrence. Mortality was nil.

Topics: Adolescent; Child; Child, Preschool; Endodermal Sinus Tumor; Epidermal Cyst; Humans; Infant; Leydig Cell Tumor; Male; Orchiectomy; Puberty; Teratoma; Testicular Neoplasms

PubMed: 9859508
DOI: No ID Found

Review

Authors: Aalia Batool, Xi-Ming Liu, Cui-Lian Zhang...

Testicular germ cell tumors (TGCTs) are generally rare but represent the most common solid tumors in young men. They are classified broadly into seminoma, which resemble primordial germ cells (PGCs), and non-seminoma, which are either undifferentiated (embryonic carcinoma) or differentiated (teratoma, yolk sac tumor, choriocarcinomas) patterning. A widespread role for microRNAs (miRNAs), in diverse molecular processes driving initiation and progression of various types of TGCTs has been recently studied. We discuss the involvement of different miRNAs in the development and progression of different types of TGCTs. Moreover, we highlight the aberrant expression of miRNAs in TGCTs and several targets, which may define miRNAs as oncomiRs or tumor suppressors. A better understanding of miRNA biology may ultimately yield further insight into the molecular mechanisms of tumorigenesis and new therapeutic strategies against TGCTs.

Topics: Animals; Biomarkers, Tumor; Carcinoma, Embryonal; Choriocarcinoma; Disease Progression; Endodermal Sinus Tumor; Gene Expression Profiling; Gene Expression Regulation; Humans; Male; MicroRNAs; Neoplasms, Germ Cell and Embryonal; Prognosis; Seminoma; Teratoma; Testicular Neoplasms; Treatment Outcome

PubMed: 30844711
DOI: 10.2741/4749

Review

Authors: Elizabeth D Euscher

Extragonadal germ cell tumors are uncommon, and although they morphologically resemble their gonadal counterparts, unexpected gonadal presentation increases the potential for erroneous diagnoses. Yolk sac tumor is a malignant germ cell tumor characterized by an extraembryonic yolk sac line of differentiation, and relative to other germ cell tumors, is characterized by varied and diverse histologic patterns. When occurring outside of typical age parameters or in extragonadal locations, the histologic variability of yolk sac tumor and its tendency to mimic somatic tumors pose diagnostic challenges. Because extragonadal yolk sac tumor of the vulva is very rare, with only isolated case reports and small series in the literature, it is often not considered in the differential diagnosis. As both prognosis and management of yolk sac tumor differ significantly from those of somatic tumors, accurate diagnosis is essential. This review discusses histologic features of extragonadal yolk sac tumor, addresses somatic tumors arising in the vulva for which yolk sac tumor may be confused, and provides guidance with respect to the use of immunohistochemistry in the diagnosis of yolk sac tumor.

Topics: Endodermal Sinus Tumor; Female; Humans; Vulvar Neoplasms

PubMed: 27959583
DOI: 10.5858/arpa.2016-0151-SA

Authors: Yanjun Hou, Bin Yang, Gloria Zhang...

CONTEXT.—

Female adnexal tumor of probable Wolffian origin (FATWO) often is a diagnostic challenge given its rarity, histologic heterogeneity, and lack of specific immunoprofile.

OBJECTIVE.—

To further understand the clinicopathologic and immunohistochemical features of this rare entity.

DESIGN.—

We studied the clinical, morphologic, and immunohistochemical features of a cohort of 11 FATWO cases from our institute.

RESULTS.—

Patients' age ranged from 25 to 76 years (mean, 55 years). Tumor size ranged from 0.5 to 18 cm (mean, 2.7 cm). Histopathologically, most tumors presented with low-grade cytologic features with low mitotic activity and lack of necrosis. Three main growth patterns were appreciated: solid, tubular, and sievelike patterns. Higher-grade nuclear atypia, increased mitotic activity, and focal necrosis were seen in 2 cases. These 2 cases were clinically considered malignant FATWO mainly because of their extra-adnexal involvement. Immunohistochemical studies found that tumor cells were positive for CD10 (11 of 11, 100%), AE1/3 (8 of 8, 100%), CAM 5.2 (4 of 5, 80%), and cytokeratin 7 (CK7; 7 of 10, 70%), and focally positive for calretinin (4 of 10, 40%), inhibin (4 of 10, 40%), epithelial membrane antigen (EMA; 3 of 9, 33%), and steroidogenic factor-1 (SF-1; 2 of 8, 25%). Lack of immunoreactivity to PAX8 and GATA3 in almost all cases indicates that FATWO is different from the tumors derived from the Müllerian or mesonephric origins. All patients with available follow-up had favorable prognosis.

CONCLUSION.—

The broad spectrum of clinical presentation, various morphologic features, and overlapping immunophenotype suggest that FATWO is a diagnosis of exclusion until it is further defined at the molecular and immunohistochemical levels.

Topics: Adenoma; Adnexal Diseases; Biomarkers, Tumor; Female; Humans; Neoplasms, Adnexal and Skin Appendage; Prognosis

PubMed: 34133728
DOI: 10.5858/arpa.2020-0432-OA

Review

Authors: Huihui Ye, Thomas M Ulbright

CONTEXT

Although relatively rare, testicular cancer is the most common solid organ malignancy in young men and remains a leading cause of cancer death in this population. Different types of testicular tumors are treated differently, with an overall very high cure rate with proper management. Pathologists must, therefore, be familiar with important diagnostic pitfalls in testicular pathology, particularly those that result in different treatments or prognoses.

OBJECTIVE

To summarize key diagnostic features and useful ancillary tools for the most frequently encountered problems in testicular tumor pathology.

DATA SOURCES

Current texts, PubMed (National Library of Medicine) articles, and archives at Indiana University School of Medicine and Beth Israel Deaconess Medical Center were all reviewed.

CONCLUSIONS

Problematic differential diagnoses include seminoma versus nonseminomatous germ cell tumors, germ cell tumors versus non-germ cell tumors, intratubular germ cell neoplasia versus atypical germ cells with maturation arrest, pseudolymphovascular invasion versus real lymphovascular invasion in germ cell tumors, and macroscopic Sertoli cell nodules versus Sertoli cell tumors. In almost all cases, awareness of the differential diagnostic possibilities based on routine light microscopic features permits application of either additional, directed observations or immunohistochemical studies that lead to an accurate diagnosis.

Topics: Carcinoma, Embryonal; Dermoid Cyst; Diagnosis, Differential; Endodermal Sinus Tumor; Epidermal Cyst; Humans; Male; Neoplasms, Germ Cell and Embryonal; Seminoma; Sertoli-Leydig Cell Tumor; Teratoma; Testicular Diseases; Testicular Neoplasms; Testis

PubMed: 22458906
DOI: 10.5858/arpa.2011-0475-RA

Review

Authors: Akram Shalaby, Veena Shenoy

CONTEXT.—

Female adnexal tumor of probable Wolffian origin (FATWO) is an extremely rare gynecologic neoplasm of low malignant potential. Fewer than 90 cases of this entity have been described in the English-language literature. It is presumed to be derived from mesonephric (Wolffian) duct remnants in the upper female genital tract. We provide a literature review to increase awareness of this extremely uncommon entity.

OBJECTIVES.—

To review the clinical and pathologic findings of FATWO and to discuss common entities in the differential diagnosis.

DATA SOURCES.—

The study involved PubMed (National Center for Biotechnology Information, Bethesda, Maryland) searches, including multiple review articles, case reports, retrospective studies, selected book chapters, and University of Mississippi Medical Center cases.

CONCLUSIONS.—

FATWO can affect patients from a wide age range and present with a nonspecific clinical presentation. It typically presents as solid tumors with occasional nodular, lobulated, or cystic appearances. FATWO can show a variety of histologic patterns which may result in diagnostic difficulties for pathologists. There is no single specific immunohistochemical stain for FATWO, and the pathogenesis and molecular alterations are not yet well understood. Although it is generally considered a benign entity, recurrent and metastatic cases have been reported. There are no current recommendations regarding the optimal clinical management of FATWO.

Topics: Adenoma; Adnexal Diseases; Female; Humans

PubMed: 31469585
DOI: 10.5858/arpa.2019-0152-RA

Authors: Soyoun Rachel Kim, Fernando Heredia, Sara Pakbaz...

Topics: Adenoma; Adnexal Diseases; Female; Humans; Middle Aged

PubMed: 34099520
DOI: 10.1136/ijgc-2020-002367

Authors: Chia-Chi Chiu, Tang-Her Jaing, Jin-Yao Lai...

Testicular neoplasms are not commonly found in children and are a formidable threat if treated inappropriately. However, there is no consensus regarding its management. This study aimed to create a holistic picture of the interprofessional team in the management of malignant testicular tumors. Seventeen patients had mixed germ cell tumors, 15 had pure yolk sac tumors, 2 had immature teratomas, 2 had teratocarcinomas, and 1 had a sex cord stromal tumor. Five lesions were diagnosed as nongerm cell tumors: 2 embryonal rhabdomyosarcomas, 2 lymphomas, and 1 acute myeloid leukemia. At initial presentation, retroperitoneal (n = 2), bone marrow (n =1), and mediastinal (n = 1) metastases were identified in 4 (10%) patients. The operative interventions performed included radical inguinal orchiectomy (n = 5), scrotal orchiectomy (n = 31), and testicular biopsy or testis-sparing enucleation of the tumor (n = 6). Postoperatively, 18 patients received either adjuvant chemotherapy (n = 14) or chemoradiation (n = 5). Five patients with mixed germ cell tumors (n = 2), group IV paratesticular rhabdomyosarcoma (n = 2), and acute myeloid leukemia with myeloid sarcoma (n =1) died of disease progression. Thirty-six patients remained alive and disease-free at the last visit. Malignant testicular tumors in children deserve proper diagnostic support from a therapeutic perspective. Any concern or suspicion of a testicular tumor warrants an inguinal approach to avoid scrotal violation.

Topics: Child; Endodermal Sinus Tumor; Humans; Male; Neoplasms, Germ Cell and Embryonal; Orchiectomy; Teratoma; Testicular Neoplasms

PubMed: 35866814
DOI: 10.1097/MD.0000000000029735