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Serous borderline tumour of the testis: Ultrasound examination shows an unilocular cyst with intracystic papillae (a). Histologically, the tumour shows papillary structures encased by a fibrous wall with cystic areas filled with clear fluid (b; x20). The tumour cells present with mild cytologic atypia and eosinophilic cytoplasm, the nuclei show a predominantly dense chromatin with prominent nucleoli (c; x40 and d; x100; e, x200; f, x200; g, x400; h, x600)
Source publication
Tumours of ovarian-epithelial type of the testis, including serous borderline tumours, represent very rare entities. They are identical to the surface epithelial tumours of the ovary and have been reported in patients from 14 to 68 years of age. We describe two cases of a 46- and a 39-year old man with incidental findings of intratesticular masses...
Contexts in source publication
Context 1
... examination of testicular sections con- firmed the cystic nature of the lesions, which were lined by a fibrous capsule (Fig. 1a) and contained residues of clear fluid. Intraluminal, irregular papillary structures lined by partially multistratified columnar cells and areas of hobnail cells could be seen. The tumour cells exhib- ited eosinophilic cytoplasm, the nuclei showed predom- inantly dense chromatin with prominent nucleoli (Fig. 1b-f ). Furthermore, there ...
Context 2
... were lined by a fibrous capsule (Fig. 1a) and contained residues of clear fluid. Intraluminal, irregular papillary structures lined by partially multistratified columnar cells and areas of hobnail cells could be seen. The tumour cells exhib- ited eosinophilic cytoplasm, the nuclei showed predom- inantly dense chromatin with prominent nucleoli (Fig. 1b-f ). Furthermore, there was mild cytological aty- pia but no mitoses (Fig. 1g + h). Psammoma bodies could not be detected. Proliferative activity revealed by Ki67 staining was below 5 % in both cases (Fig. 2a). Immunohistochemical examination of the tumour cells displayed expression of pan-cytokeratin AE3 (Fig. 2b), estrogen-and ...
Context 3
... fluid. Intraluminal, irregular papillary structures lined by partially multistratified columnar cells and areas of hobnail cells could be seen. The tumour cells exhib- ited eosinophilic cytoplasm, the nuclei showed predom- inantly dense chromatin with prominent nucleoli (Fig. 1b-f ). Furthermore, there was mild cytological aty- pia but no mitoses (Fig. 1g + h). Psammoma bodies could not be detected. Proliferative activity revealed by Ki67 staining was below 5 % in both cases (Fig. 2a). Immunohistochemical examination of the tumour cells displayed expression of pan-cytokeratin AE3 (Fig. 2b), estrogen-and progesterone receptor ( Fig. 2c-d), Wilms' tumor protein (WT1), and PAX8 (Paired box gene ...
Citations
... В сомнительных случаях иммуногистохимическое исследование позволяет уточнить гистологический тип опухоли и провести дифференциальный диагноз с другими новообразованиями, включая мезотелиальные опухоли. Для серозной пограничной опухоли яичка характерны наличие окрашивания в клетках опухоли таких иммуногистохимических маркеров, как WT1, PAX-8, BerEP4, ER, PR, CA125, CEA, CK5/6 и CK7, а также низкие показатели индекса Ki-67 [1,7,10,11], в то время как в опухолях мезотелиального происхождения определяется окрашивание калретинина, подопланина и тромбомодулина. Также следует включать в дифференциально-диагностический ряд редко встречающуюся аденокарциному сети яичка, для которой не характерны кистозное строение и наличие сосочковых структур. ...
Serous tumors are rare in the group of non-germ cell testicular tumors, ad are morphologically similar to serous ovarian tumors. Preoperative diagnosis of this tumor is difficult due to the lack of specific signs according to clinical and instrumental research methods. In turn, histological verification of a serous borderline tumor is also a difficult task and requires differential diagnosis, primarily with serous cystadenocarcinoma, which, unlike a serous borderline tumor, can metastasize and is generally characterized by worse prognosis. Immunohistochemical examination allows to exclude other tumors with a similar morphological structure, including mesothelial tumors. Due to the rare occurrence of testicular serous tumors, there is minimal clinical experience in the treatment of these tumors worldwide, and there is still no consensus on guidelines for their treatment. Radical orchiectomy is recommended for patients with border-line serous tumors.
This article presents a clinical case of a testicular serous borderline papillary tumor with a discussion of the literature data on the instrumental, morphological, and immunohistochemical characteristics of this rare tumor in men.
... Diagnosis is based on histology, which describes a simple cyst wall structure composed of columnar ciliated cells expressing vimentin and cytokeratin as demonstrated in Figure 2. They concurrently lack Wolffian Duct CD10 and calretinin expression, which are consistent with fetal Mullerian duct structures [9]. The rarity of this tumor places it low on a differential diagnosis, in favor of more common entities. ...
Paratesticular cystadenomas remain a very rare entity, typically presenting as a painless mass, often indistinguishable from the testicle. As such, the predominant management seems to be complete excision via various approaches, which often proves curative. Given its rarity, post-operative surveillance has not been standardized; most patients and providers elect a more conservative surveillance approach. Based on the available literature, this seems appropriate, given the lack of morbidity or recurrence associated with these types of tumors.
... Other differential diagnoses include primary testicular tumors, such as mesothelioma, adenocarcinoma of the rete, appendix testis, epididymis, and germ cell tumors. (6,16,24) Mesothelioma of the tunica vaginalis can be excluded by histological differences, such as a lack of psammoma bodies, low cellularity, intestinal type, and ciliated serous morphology, as well as the immunohistochemical profile. (6,24) The possible diagnosis of adenocarcinoma of the rete testis, appendix testis, or epididymis can be eliminated by the parenchymal location of the tumor and the specific histological features. ...
... (6,16,24) Mesothelioma of the tunica vaginalis can be excluded by histological differences, such as a lack of psammoma bodies, low cellularity, intestinal type, and ciliated serous morphology, as well as the immunohistochemical profile. (6,24) The possible diagnosis of adenocarcinoma of the rete testis, appendix testis, or epididymis can be eliminated by the parenchymal location of the tumor and the specific histological features. (6) These lesions are located in the extra scrotal region, and their histological characteristics include tubular and tubule-papillary structures lined by cuboidal cells. ...
Primary mucinous cystadenocarcinoma (MCA) of the testis is extremely rare, only 10 cases having been reported to date. Metastases of mucinous adenocarcinomas that have originated in different sites can mimic primary MAC and must be included in the differential diagnosis. We report a case of primary MCA of the tunica vaginalis testis in a 28-year-old patient who presented with a painless mass on the left side of the scrotum. We present the clinical and pathological characteristics to contribute to the further understanding of these rare tumors.
... On IHC, tumors cells show positive stains for CK7, PAX8, WT1, p16, and ER and negative stains for CK20 and Napsin A antibodies [2]. P53 gene expression, although rarely performed, appears to be aberrant in almost all cases of high-grade serous cystadenocarcinomas [15], and the BRAF V600E mutation, commonly seen in low-grade ovarian epithelial tumors, is recently reported in a case of a borderline serous tumor of the testis [16]. ...
Background
Germ cell tumors account for more than 95% of testicular cancers followed by sex-cord stromal tumors. The occurrence of serous cystadenocarcinoma in testicular and paratesticular tissue is very rare with distinct clinical behavior.
Case presentation
Herein, we report the case of a 39-year-old man who presented with right testicular swelling. Anatomopathological examination of the right orchiectomy revealed a high-grade paratesticular serous cystadenocarcinoma. Long-term follow-up with close monitoring was adopted in the absence of concomitant metastasis. The patient was disease-free at two years post-surgery.
Conclusion
Owing to its scarcity, there are no standardized guidelines for optimal management of this histological entity. Sharing case reports provide valuable information to support clinical decisions.
... However, they identified PIK3CA, KRAS and MAP3K1 mutations, suggesting involvement of PI3-AKT and MAPK signaling (21)(22)(23). Although BT are almost exclusive ovarian neoplasms, they may constitute a highly unusual tumor of the testis (24). It remains a matter of debate whether ovarian-type epithelial tumors of the testis originate from the remnants of Müllerian ducts in the paratesticular connective tissue, epididymis and spermatic cord, or from Müllerian metaplasia of the mesothelium of the tunica vaginalis testis (11). ...
Malignant Brenner Tumor (mBT) is extremely rare. Although BT are almost exclusive ovarian neoplasms, they may constitute a highly unusual tumor of the testis; in fact, only seven fully documented cases have been reported to date. Because of their rarity, the pathogenesis of these tumors has not been clarified and there is no standard therapeutic approach. We report the first case of epididymal mBT with synchronous, multiple, liver metastases and a very dramatic clinical course. Both primary tumor and metastasis were subjected to mutational analysis of 20 cancer associated genes. Primary tumor showed FGFR3 Tyr375Cys and PIK3CA His1047Arg missense mutations. Both mutations are reported as pathogenic in ClinVar database. The same FGFR3 mutation was present in liver metastasis. Based on these results we believe that the FGFR pathway could be an ideal candidate for personalized treatment, offering hope to a subset of patients with mBT. Personalized approach, including mutational analysis and molecular testing should be required in patients with rare tumors in order to clarify diagnosis and improve therapeutic strategies.
... Indeed, several hypotheses are proposed [12]; these tumors may arise from (1) embryological Mullerian remnants in paratesticular tissue, spermatic cord, or epididymis or (2) Mullerian metaplasia of intratesticular inclusions of the tunica vaginalis. This second hypothesis shows a similarity with the origin of the development of the ovarian counterpart, which seems to grow from germinal surface epithelium [13]. ...
... However, the mutational profile of such testicular tumors is poorly understood, probably due to the rarity of these entities [9,12,14]. ...
... This is the second reported case of a KRAS gene mutation in this type of testis tumor. The other gene mutations in these tumors include BRAF gene mutations (Table 2) [9,12,14]. ...
Ovarian-like epithelial tumors of the testis, including serous borderline tumors, are rare entities. We report the case of a 60-year-old man with a left intratesticular mass who had a radical orchidectomy. Histologically, the tumor was identical to the ovarian counterpart showing a well-delineated cystic lesion characterized by intraluminal papillae. The papillae are lined by atypical cuboidal or ciliated cells and are associated with psammoma bodies. The tumor cells express cytokeratin 7 (CK7), cytokeratin 5-6 (CK5-6), cancer antigen 125 (CA125), estrogen (ER), progesterone (PR), Wilm's tumor gene (WT1), paired box gene 8 (PAX8), Ber-EP4, and epithelial membrane antigen (EMA). The diagnosis of a serous borderline tumor of the testis was proposed. Mutation testing using next-generation sequencing showed a Q61K KRAS gene mutation. To the best of our knowledge, this is the second case report of a serous borderline tumor of the testis with a Q61K KRAS gene mutation.
... To date, around 52 cases of serous type tumors of testis are documented in the world literature. [1,2] There are no consensus guidelines on the recommended treatment of these tumors. Therefore, we report borderline serous papillary tumor of the testis in a 37-year-old male, which was clinically suspected to be a testicular malignancy. ...
... They may present as unilocular or multilocular cystic masses which have intracystic rounded branching papillae with fibrovascular core and lined by cuboidal to columnar epithelium exhibiting nuclear atypia and occasional mitoses. [1,2] Our patient presented with unilocular cyst with papillary excrescences. IHC can be used as an adjunct to exclude histological differential diagnoses. ...
... Absence of stromal invasion excluded the possibility of low-grade serous carcinoma. [1,[3][4][5][6] Several hypotheses exist regarding the histogenesis of epithelial tumors in testicular tissue. Some authors suggest that tumors may arise from the remnants of Mullerian ducts that form male appendix, testis, epididymis, and connective tissue between testis and epididymis and spermatic cord. ...
Ovarian epithelial type tumor of the testis is a rare entity. Herein, we report borderline serous papillary tumor of the testis in a 37-year-old male, which was clinically suspected to be a testicular malignancy.
... The immunohistochemical markers have reported to be positive for CA125, Cytokeratin 7, CD 15, epithelial membrane antigen and Ber-EP4 (2,3). ...
... Although these markers are not very specific they help rule out other differential diagnoses like malignant mesothelioma (3,4). ...
... Since the diagnosis of ovarian epithelial type carcinomas is difficult based on the morphological similarities(5), other diagnoses such as malignant mesothelioma of the tunica vaginalis of testis should also be considered, as these tumors may be microscopically similar to ovarian type epithelial tumors and they can be ruled out with the help of immunohistochemically markers like calretinin (3,7). ...
Ovarian epithelial type carcinomas of testis are an extremely rare group of tumors, a few cases of which having been reported. We present the case of a 67-year-old man, presented with testicular mass and inflation, who underwent radical orchiectomy and pathological and immunohistochemical assessments revealed serous papillary carcinoma of ovarian epithelial type tumor of testis.
... En 1986, Young et Scully ont étudié 3 cas de TSBO et se sont basés sur 11 cas de la littérature antérieure [1]. La plupart de ces TSBO sont décrites dans le tissu paratesticulaire [2] et parfois en localisation intra-testiculaire [3]. Quelle que soit la localisation, l'aspect morphologique de ces tumeurs est identique à celle des cystadénomes séreux classiques de l'ovaire. ...
... On peut y observer des psamomes. En immunohistochimie, les cellules tumorales expriment CK7, EMA, RE, RP et PAX8 à la différence des anticorps CK20, calrétinine, CD10, CDX2 et CK5/6 [2,3]. ...
... D'un point de vue physiopathologique, deux hypothèses sont actuellement privilégiées. Ces tumeurs se développeraient soit à partir des résidus de canaux de Müller présents dans l'appendix testis, le tissu para-testiculaire, le cordon spermatique ou l'épididyme, soit à partir d'une métaplasie Müllerienne de la vaginale [3]. L'apparition de ces tumeurs au sein même du parenchyme testiculaire surviendrait sur des inclusions mésothéliales métaplasiques [4]. ...
... The immunohistochemical profile of testicular serous tumors is virtually indistinguishable from that of ovarian serous tumors [18,19]. Most cases are cytokeratin (CK)7-positive, and CK20-negative, and also display expression of other epithelial markers, including CAM5.2, AE1/AE3, Ber-EP4, and MOC-31 [6,36]. ...
... Most cases are cytokeratin (CK)7-positive, and CK20-negative, and also display expression of other epithelial markers, including CAM5.2, AE1/AE3, Ber-EP4, and MOC-31 [6,36]. In addition, consistent positivity for markers of Mullerian differentiation, such as PAX-8, WT-1, estrogen receptor (ER), progesterone receptor (PR), and CA-125, has been observed [5,18]. Some cases also exhibit reactivity to CA19-9, carcinoembryonic antigen (CEA), S100, and Leu-M1 (CD15) [19,20]. ...
... Recent studies on ovarian-type testicular tumors have focused on molecular characterization and the role of adjuvant therapy in treatment, particularly for serous tumors. In the past few years, several studies demonstrated the presence of the BRAF V600E mutation in SBTs of the testis, also seen in SBTs and low-grade serous carcinomas of the ovary [18,28,107]. This finding provides a clear genetic association between testicular and ovarian low-grade serous neoplasms and suggests similar pathogenesis to ovarian SBTs and low-grade serous carcinomas believed to derive from Mullerian metaplasia of surface epithelium [28]. ...
Ovarian-type epithelial tumors rarely occur in the human testis and paratesticular region. Histologically, these tumors closely resemble their ovarian counterparts and are therefore classified similarly to ovarian epithelial tumors. Most reported ovarian-type tumor cases are serous tumors, but the full spectrum of ovarian neoplasms has been described, including mucinous, endometrioid, clear cell, and Brenner tumors. In this review, we describe the clinical, morphologic, and immunohistochemical features of Mullerian-type epithelial tumors seen in the testis, with an emphasis on comparison with ovarian tumors. We also discuss theories of pathogenesis, considerations in the differential diagnosis, and recent advances in molecular characterization and therapeutic modalities.
