Carcinoma of rete testis is an extremely rare malignant tumor arising from its epithelium. Prognosis is poor with mean survival of 8 months. Lymph node metastases and the size of the tumor larger than 5 cm are poor prognostic factors. We report a case of primary undifferentiated carcinoma of the rete testis in a 46-year-old man who presented with testicular enlargement without previous trauma or cryptorchidism, and with extensive peritoneal carcinomatosis, retroperitoneal lymph node metastases and fatal outcome. We present this case because of the rarity of the carcinoma of the rete testis and its challenging diagnosis.

A 31-year-old man with left-sided testicular pain lasting a couple of months was referred to our urology department due to a suspected testicular tumor. Physical examination showed a hard, thickened, and small left testis on palpation with a diffuse, inhomogeneous ultrasonographic appearance. After a urologic examination, a left-sided inguinal orchiectomy was performed. The testis, epididymis, and spermatic cord were sent to pathology. Gross examination revealed a cystic cavity filled with brown fluid and the surrounding brownish parenchyma measuring up to 3.5 cm in diameter. Histologic examination showed a cystically dilated rete testis lined with cuboidal epithelium and a positive immunohistochemical reaction to cytokeratins. Microscopically, the cystic cavity was a pseudocyst filled with extravasated erythrocytes and abundant clusters of siderophages. The siderophages extended into the testicular parenchyma, surrounding the seminiferous tubules and spreading out around the ducts of the epididymis, which were also cystically dilated with siderophages inside their lumina. On the basis of clinical data, histological, and immunohistochemical analysis, the patient was diagnosed with cystic dysplasia of the rete testis. The literature shows an association between cystic dysplasia of the rete testis and ipsilateral genitourinary anomalies. Therefore, our patient underwent a multi-slice computed tomography scan, which revealed ipsilateral renal agenesis, a right seminal vesicle cyst reaching up to the iliac arteries, and a multicystic formation cranial to the prostate.

Adenocarcinoma of the rete testis is a rare malignant tumor with poor prognosis. We report a case of adenocarcinoma of the rete testis. A 55-year-old man became aware of discomfort in the right scrotum. Negative results were obtained for the serum markers AFP, β-human chorionic gonadotropin (β-HCG), and LDH. Computed tomography (CT) showed enhancement of the right testis. Radical orchiectomy was performed. Immunohistochemical examination of the resected specimen showed positive results for CEA, and adenocarcinoma of the rete testis was diagnosed. Serum CEA level was elevated. CT showed swelling of the para-aortic lymph nodes. Retroperitoneal lymph node dissection (RPLND) was performed, and serum CEA then normalized. The patient developed penile metastases 4 months after RPLND, and serum CEA level again increased. Total penile resection was performed. TIP (Paclitaxel, Ifosfamide, Cisplatin) therapy was started after lung metastasis and increased serum CEA were identified. CT after 2 cycles of TIP therapy revealed disappearance of lung metastasis and normalization of serum CEA. Five months later, CT showed recurrence of lung metastases.

BACKGROUND: Adenocarcinoma of the rete testis (AORT) is an extremely rare malignant tumor with poor prognosis and limited responsiveness to traditional chemotherapy. Few previous studies have focused on the molecular mechanisms underlying therapy resistance in AORT and further scrutiny is required to enable searches for targeted drugs to guide treatment selection.
METHODS: The current case concerns a 55-year-old man with AORT who presented with isolated bone metastasis at initial diagnosis and experienced rapid disease progression after multi-line platinum-based combination chemotherapy. Next-generation sequencing revealed a novel somatic lysine methyltransferase 2C (KMT2C) c.5605 T > C mutation in exon 36 with an abundance of 49.27%. The patient received antiangiogenic drug treatment for 2 months but this was discontinued due to unacceptable anorexia and nausea. He survived for 12 months after diagnosis.
CONCLUSIONS: A potential correlation between AORT primary multi-drug resistance and KMT2C mutations is implied. Further studies are needed to determine the efficacy of PARP1/2 inhibitors for tumors with KMT2C mutations.

Cystic dysplasia of the rete testis (CDRT) is a rare cause of testicular masses in children. The pathogenesis of this malformation remains unclear. It is often associated with other genitourinary anomalies, commonly presenting as agenesis or dysplasia of the ipsilateral kidney. A case involving a 9-year-old boy with a testicular lesion and ipsilateral renal agenesis, who was diagnosed with CDRT after histological examination, is reported. In addition, a systematic review of the literature was performed to better understand this pathology to design the most appropriate treatment and follow-up strategy for patients with CDRT.

Adenocarcinoma of the rete testis is an extremely rare and aggressive tumor that carries a poor prognosis. Successful long-term treatment for such tumors remains elusive as more cases are discovered worldwide. Treatment typically involves radical orchiectomy, retroperitoneal pelvic lymph node dissection, adjuvant chemotherapy, and/or continued surveillance. Here we describe the case of a 42-year-old male with a history of low testosterone who presented with a localized adenocarcinoma of the left rete testis. He was treated with radical orchiectomy and continued surveillance alone due to a lack of evidence of metastasis on follow-up imaging. History, prognosis, diagnostics, and treatment guidelines, as well as the most significant recent cases since the last rete testis adenocarcinoma literature meta-analysis, are discussed.

Cystic dysplasia of the rete testis is a rare benign abnormality seen in the pediatric population. Diagnosed by scrotal ultrasonography, this lesion is often associated with congenital renal tract anomalies, most commonly ipsilateral renal agenesis or multicystic dysplastic kidney. Treatment traditionally involves orchiectomy or testicular sparing surgery; however, conservative treatment with surveillance has been used as an appropriate alternative. We present the case of a 5-year-old boy with cystic dysplasia of the rete testis with associated unilateral renal agenesis.

Primary adenocarcinoma of the rete testis, which arises from the collecting systems of the testis, is an extremely rare tumor with a poor prognosis. A case of primary adenocarcinoma of the rete testis with elevation of serum carcinoembryonic antigen (CEA) levels is reported. A 59-year-old man presented with left scrotal swelling. Ultrasonography showed a left hydrocele and swelling of the left testis with calcification. Computed tomography showed para-aortic lymph node swelling and multiple lung metastases. Germ cell tumor markers were within the normal range. Radical orchiectomy was performed, and histological examination showed adenocarcinoma. The tumor cells were immunohistochemically positive for CEA. There was no evidence of other primary carcinomas. The tumor was finally diagnosed as a primary adenocarcinoma of the rete testis. The patient had bone metastases 8 months after orchiectomy with increased serum CEA levels. CEA might be a marker for primary adenocarcinoma of the rete testis.

Sertoliform cystadenoma is a rare benign tumor of the rete testis with 8 previously reported cases and an additional 14 cases reported in an abstract form. It usually presents with a unilateral scrotal mass, clinically and radiologically indistinguishable from malignant testicular tumors. We report a 39-year-old man who presented with a right testicular mass. The patient underwent radical inguinal orchiectomy. Grossly, no masses were appreciated. After histologic examination with subsequent immunohistochemical staining, a sertoliform cystadenoma of the rete testis was diagnosed.

BACKGROUND: Adenocarcinoma of rete testis is an extremely rare dia-gnosis described in around 70 patients worldwide. The prognosis of the disease in metastatic stage is very poor and there is no standard systemic treatment available.
METHODS: Herein we present a unique case report of a 47-year- old man with metastatic adenocarcinoma of rete testis who achieved substantial disease response after four cycles of paclitaxel, ifosfamide and cisplatin. The chemotherapy was administered in five -day regimen, which comprised 250 mg/ m2 of paclitaxel on day one, 20 mg/ m2 of cisplatin on day one to five and 1,2 g/ m2 of ifosfamide on day one to five, in a three-week interval. The patient received prophylactic pegfilgrastim after each cycle of TIP. The treatment was well tolerated -  without any significant toxicity.
RESULTS: Patient achieved a partial 14- month remission.
CONCLUSIONS: On basis of this experience we suggest that paclitaxel, ifosfamide and cisplatin might be adopted as novel agents in treatment of rete testis adenocarcinoma.