Dedifferentiated liposarcoma of the spermatic cord.

A man in his 60s presented to an outside hospital with persistent groin pain and a scrotal mass which was thought to be a recurrent hernia. Three months after initial presentation, the patient was found to have dedifferentiated liposarcoma (LPS) of the spermatic cord. LPS of the spermatic cord is a rare entity; however, clinicians should have LPS on the differential diagnosis especially in men with recurrent scrotal pain and mass. If unrecognised, LPS is associated with a high degree of morbidity and mortality. LPS can be subdivided into well-differentiated LPS, dedifferentiated LPS, myxoid LPS and pleomorphic LPS. In patients with advanced or metastatic LPS, chemotherapy consisting of Adriamycin, ifosfamide and mesna is used despite LPS being relatively chemoresistant. Therapies inhibiting mouse double minute 2 homologue, an oncoprotein that is a negative regulator of the tumour suppressor p53, appear to be promising in preclinical trials.

Liposarcoma of spermatic cord mimicking an inguinal hernia A case report and review of the literature.

AIM: Liposarcoma of the spermatic cord (LSC) is a tumour often mistaken for common inguinal swelling as hernia and the aim of this work is to present our case with a review of the Literature to define the management of this rare condition. MATERIAL OF STUDY: A systematic review has been realised, considering English language articles published on Pubmed, between 1956 and 2022, using as key words "Liposarcoma of the spermatic cord". RESULTS: 160 studies described 420 cases of LSC and in 40 cases the patient had undergone surgery with an initial diagnosis of inguinal hernia. DISCUSSION: LSC is a very rare entity of genitourinary malignancies, occurring more often in the spermatic cord and diagnosis can be difficult. Our case and Literature data confirm the role of imaging in not conventional inguinal swelling, to avoid diagnostic mistakes and to define preoperatively the correct surgical management. CONCLUSIONS: Imaging is mandatory in case of diagnostic doubt. The recommended treatment is a radical high orchiectomy with clear margins. A long follow-up period is necessary to detect a local recurrence which may occur even several years after the primary therapy. KEY WORDS: Inguinal swelling, Liposarcoma, Spermatic cord.

Extremely rare pediatric primary scrotum tumor: spermatic cord hemolymphangioma for a case report and literature review.

Hemolymphangioma is an uncommon benign tumor type that commonly occurs in the head and neck. Primary spermatic cord hemolymphangioma (SCH) with only several reported, however, is extremely rare. Clinical diagnosis can be challenging because of its rarity. Although spermatic cord hemolymphangiomas are benign tumors, there is still a high recurrence rate in postoperative. A 15-year-old boy presented to our hospital with complaints of scrotal for 15 days and did not have other associated symptoms. The male genital color Doppler ultrasound revealed that a cystic echo in the left spermatic cord region and above the testes was about 32 mm × 20 mm × 14 mm. He underwent left en bloc scrotum tumor resection under general anesthesia, and pathologic examination showed SCH. He was discharged from the hospital in the second postoperative day. After 1-month follow-up, the patient recovered well without recurrence. The patient is currently in follow-up phase. Up to date, only a few cases have been reported in the literature about SCH. So, we hope to raise the awareness of the diagnosis of SCH in clinical practice although this case.

A case of spermatic cord B-cell lymphoma relapsing to the brain in a dog.

A 13-year-old, intact male mixed-breed dog was referred to our clinic for lethargy and asthenia following an episode of gastroenteritis. As an incidental finding during abdominal ultrasound, a mass on the right spermatic cord was seen. Cytology of the mass revealed a monomorphic population of large, round cells with a lymphoid appearance. A bilateral orchiectomy was conducted, and histopathology revealed the presence of a B-cell lymphoma in the right spermatic cord. Based on clinical staging, which showed no involvement of other sites, no additional treatment was administered. Recheck evaluations were scheduled for every 3 mo thereafter. Five months after surgery, the dog developed left central vestibular syndrome with a paradoxical right-sided head tilt. An MRI of the brain showed multifocal lesions and, due to a rapidly worsening clinical condition, the dog was humanely euthanized. The histopathology of the brain lesions was consistent with B-cell lymphoma. Key clinical message: This is the first report of a primary spermatic cord lymphoma relapsing to the brain in a dog. Although rare, spermatic cord tumors should be included among the differential diagnoses for masses arising from the spermatic cord. If lymphoma is diagnosed, location to other sites, especially to the central nervous system, should be considered.

Un cas de lymphome à cellules B du cordon spermatique récidivant au cerveau chez un chien. Un chien de race mixte mâle intact de 13 ans a été référé à notre clinique pour léthargie et asthénie à la suite d'un épisode de gastro-entérite. Comme découverte fortuite lors d'une échographie abdominale, une masse sur le cordon spermatique droit a été observée. La cytologie de la masse a révélé une population monomorphe de grosses cellules rondes d'aspect lymphoïde. Une orchidectomie bilatérale a été réalisée et l'histopathologie a révélé la présence d'un lymphome à cellules B dans le cordon spermatique droit. Sur la base du stade clinique, qui n'a montré aucune implication d'autres sites, aucun traitement supplémentaire n'a été administré. Des évaluations de contrôle étaient programmées tous les 3 mois par la suite. Cinq mois après la chirurgie, le chien a développé un syndrome vestibulaire central gauche avec une inclinaison paradoxale de la tête du côté droit. Une IRM du cerveau a montré des lésions multifocales et, en raison d'une détérioration rapide de l'état clinique, le chien a été euthanasié sans cruauté. L'histopathologie des lésions cérébrales correspondait à un lymphome à cellules B.Message clinique clé :Il s'agit du premier rapport d'un lymphome primaire du cordon spermatique récidivant au cerveau chez un chien. Bien que rares, les tumeurs du cordon spermatique doivent être incluses dans les diagnostics différentiels des masses provenant du cordon spermatique. Si un lymphome est diagnostiqué, la localisation vers d'autres sites, en particulier vers le système nerveux central, doit être envisagée.(Traduit par Dr Serge Messier).

Arteriovenous malformation of the spermatic cord mimicking reducible inguinal hernia.

Arteriovenous malformations are a vascular anomaly most often found in the central nervous system; however, they can present ubiquitously in any organs or tissues. We present the case of a 55-year-old man who developed a tender, reducible inguinal bulge and underwent laparoscopic transabdominal preperitoneal inguinal hernia repair under the clinical diagnosis of an inguinal hernia. Intraoperative observation revealed no hernia sac, but a poorly defined spermatic cord mass, appearing to be responsible for the patient's symptoms, was found and removed. The pathology of the mass was consistent with the diagnosis of an arteriovenous malformation of the spermatic cord. Surgeons should keep in mind the small possibility of arteriovenous malformations in patients with clinical presentation of an inguinal hernia, as they may cause massive bleeding during and after the operation unless handled appropriately.

Rare paratesticular localization of dedifferentiated liposarcoma: Case report and review of the literature.

RATIONALE: Dedifferentiated liposarcoma is defined as a malignant tumor that changes its shape from a well-differentiated liposarcoma to a non-liposarcomatous form. Most paratesticular liposarcomas manifest as an inguinal, painless, slow-growing mass. The standard treatment is extensive surgical excision, radiotherapy being proposed for cases with positive margins, those with recurrence, or in cases of the existence of unfavorable prognostic factors. PATIENT CONCERNS: We present the case of a young patient diagnosed initially with left hydrocele, which after 2 years proved to mask a differentiated liposarcoma of the spermatic cord. The initial clinical manifestations were represented by the increase in volume of the left groin-scrotal region and pain at this level. DIAGNOSIS: Microscopic examination in hematoxylin-eosin staining highlighted the presence of lipoblasts and fibroblasts in association with areas of hemorrhage and tumor necrosis. The performed immunohistochemical tests confirmed the diagnosis of dedifferentiated liposarcoma. To support and confirm the presence of the mouse double minute 2 homolog gene mutation, chromogenic in situ hybridization analysis was performed. INTERVENTIONS: The initial treatment was the surgical one. After 2 weeks, the patient received zolendronic acid for hypercalcemia which was caused by the osseous metastasis. OUTCOMES: The patient died secondary to acute renal failure caused by hypercalcemia despite the treatment received. LESSONS: This case underlines the importance of both the correct management of oncological patients, as well as immunohistochemical and genetic tests in the identification of prognostic factors, with the ultimate goal of administering an appropriate oncological treatment.

Prognostic factors and outcomes of adult spermatic cord sarcoma. A study from the French Sarcoma Group.

PURPOSE: To evaluate the outcomes of adult patients with spermatic cord sarcoma (SCS). METHODS: All consecutive patients with SCS managed by the French Sarcoma Group from 1980 to 2017 were analysed retrospectively. Multivariate analysis (MVA) was used to identify independent correlates of overall survival (OS), metastasis-free survival (MFS), and local relapse-free survival (LRFS). RESULTS: A total of 224 patients were recorded. The median age was 65.1 years. Forty-one (20.1%) SCSs were discovered unexpectedly during inguinal hernia surgery. The most common subtypes were liposarcoma (LPS) (73%) and leiomyosarcoma (LMS) (12.5%). The initial treatment was surgery for 218 (97.3%) patients. Forty-two patients (18.8%) received radiotherapy, 17 patients (7.6%) received chemotherapy. The median follow-up was 5.1 years. The median OS was 13.9 years. In MVA, OS decreased significantly with histology (HR, well-differentiated LPS versus others = 0.096; p = 0.0224), high grade (HR, 3 versus 1-2 = 2.7; p = 0.0111), previous cancer and metastasis at diagnosis (HR = 6.8; p = 0.0006). The five-year MFS was 85.9% [95% CI: 79.3-90.6]. In MVA, significant factors associated with MFS were LMS subtype (HR = 4.517; p < 10-4) and grade 3 (HR = 3.664; p < 10-3). The five-year LRFS survival rate was 67.9% [95% CI: 59.6-74.9]. In MVA, significant factors associated with local relapse were margins and wide reresection (WRR) after incomplete resection. OS was not significantly different between patients with initial R0/R1 resection and R2 patients who underwent WRR. CONCLUSIONS: Unplanned surgery affected 20.1% of SCSs. A nonreducible painless inguinal lump should suggest a sarcoma. WRR with R0 resection achieved similar OS to patients with correct surgery upfront.

Spermatic cord leiomyosarcoma in a young male: A case report and literature review.

Spermatic cord Leiomyosarcoma is an extremely rare intrascrotal tumour. Owing to its rarity, no definitive management guidelines have been formulated as yet. The majority of published literature comprises of case reports or case series and show varying outcomes depending upon multiple patient- and disease-related factors. Almost all cases are older adults with majority in the sixth or seventh decades of life. It is commonly labelled as an indolent curable tumour if treated Spermatic cord Leiomyosarcoma is an extremely rare intrascrotal tumour. Owing to its rarity, no definitive management guidelines have been formulated as yet. The majority of published literature comprises of case reports or case series and show varying outcomes depending upon multiple patient- and disease-related factors. Almost all cases are older adults with majority in the sixth or seventh decades of life. It is commonly labelled as an indolent curable tumour if treated early by radical orchiectomy. The role of lymphadenectomy, adjuvant radiotherapy or chemotherapy is unclear. This case report concerns a young 38-year-old man who suffered from a painless firm left hemiscrotal mass for the past two years. Ultrasonography showed an intrascrotal paratesticular mass. Metastatic workup was negative. Left radical orchiectomy was performed and histopathology of the surgical specimen revealed leiomyosarcoma of the spermatic cord. The patient is on post-surgery follow-up and disease-free for six months. A literature review is also presented.

Cremaster muscle thickening: the anatomic difference in men with testicular retraction due to hyperactive cremaster muscle reflex.

The objective was to assess whether men suffering from testicular retraction secondary to hyperactive cremaster muscle reflex have an anatomic difference in the thickness of the cremaster muscle in comparison to men who do not have retraction. From March 2021 to December 2021, 21 men underwent microsurgical subinguinal cremaster muscle release (MSCMR) on 33 spermatic cord units, as 12 of them had bilateral surgery, at Surgicare of South Austin Ambulatory Surgery Center in Austin, TX, USA. During that same time frame, 36 men underwent subinguinal microsurgical varicocele repair on 41 spermatic cord units, as 5 were bilateral for infertility. The thickness of cremaster muscles was measured by the operating surgeon in men undergoing MSCMR and varicocele repair. Comparison was made between the cremaster muscle thickness in men with testicular retraction due to a hyperactive cremaster muscle reflex undergoing MSCMR and the cremaster muscle thickness in men undergoing varicocele repair for infertility with no history of testicular retraction, which served as an anatomic control. The mean cremaster muscle thickness in men who underwent MSCMR was significantly greater than those undergoing varicocele repair for infertility, with a mean cremaster muscle thickness of 3.9 (standard deviation [s.d.]: 1.2) mm vs 1.0 (s.d.: 0.4) mm, respectively. Men with testicular retraction secondary to a hyperactive cremaster muscle reflex demonstrate thicker cremaster muscles than controls, those undergoing varicocele repair. An anatomic difference may be a beginning to understanding the pathology in men who struggle with testicular retraction.

Total embedding of spermatic cord and hilar soft tissue in orchiectomy for seminoma: does the extensive sampling improve pathologic risk factors?

Pure seminomas represent the majority of testicular germ cell tumors and accurate diagnosis and staging require an accurate sampling of radical orchiectomy specimens. The aim of our study is to find the most informative gross sampling method for orchiectomy specimens. We performed the extensive sampling of 88 radical orchiectomy specimens embedding in their entirety testicular hilum, rete testis, hilar soft tissue, and spermatic cord. We examined the impact of this procedure on tumor stage, prognostic parameters (lymphovascular invasion and infiltration of rete testis, epididymis, tunica vaginalis, and spermatic cord), and their relationship with recurrence. Eighty-eight seminomas from 88 radical orchiectomies were sampled. Seventy-seven cases (87.5%) presented as clinical stage I and 11 cases (12.5%) as clinical stage II. The follow-up period range was 18-54 months and 82 patients (93.2%) had a minimum of 2-year follow-up. Tumor size ranged from 0.4 to 16 cm (mean 3.6) requiring a mean of 7.1 sections for entire tumoral sampling. Epididymis required 2 to 8 sections (mean 3.3), and hilum and hilar soft tissues 2 to 9 sections (mean 3.4). Epididymal infiltration and lymphovascular invasion resulted significant at multivariate analysis generating a receiver operating characteristic (ROC) curve with area under curve of 0.778. All the other parameters (except for pagetoid rete testis infiltration) were significant to predict metastasis only at univariate analysis. Extensive sampling of radical orchiectomy specimens does not improve the accuracy of staging in pure seminomas. Lymphovascular invasion and epididymal infiltration are useful to predict metastasis.

CT imaging of an unusual malignant inguinal mass: metastatic parafunicular dedifferentiated liposarcoma.

A man in his 50s was redirected by his general practitioner to our University Hospital for a radiological evaluation after the complaint of a left inguinal swelling. The preliminary ultrasound and CT scan showed a left parafunicolar mass located between the root of the penis and the spermatic cord. The lesion was biopsied shortly after and histologically diagnosed as a dedifferentiated liposarcoma. The patient underwent a cycle of neoadjuvant radiotherapy, which proved to be ineffective. He was then subjected to an organ sparing surgical removal of the mass, followed by a second organ sparing removal of some residual disease. The last CT scan showed absence of the inguinal disease; however, lung and hepatic metastasis were detected, which are scheduled to be treated with a course of chemotherapy and radiofrequency.

Hydrocele with Paratesticular Liposarcoma.

Paratesticular liposarcoma (PLS) is a rare type of genitourinary malignancy in the spermatic cord presenting as scrotal swelling. In this case study, we report an 82-year male who presented with prolonged left scrotal swelling and pain that did not respond to analgesic treatment for one week. His ultrasound confirmed increased fluid content in the left scrotal compartment in the form of septated hydrocele, and hydrocelectomy was planned. During the operation, infected fluid was drained. The testicle and surrounding tissues were infected; hence, left orchiectomy was performed. The pathological examination revealed a dedifferentiated liposarcoma. To our knowledge, this is the first report of liposarcoma of the spermatic cord with hydrocele in the literature. Key Words: Liposarcoma, Spermatic cord, Scrotal swelling, Hydrocele.

Leiomyosarcoma of the spermatic cord: a rare paratesticular neoplasm case report.

BACKGROUND: Primary soft tissue sarcomas contribute to only 2% of all malignancies arising from the male genitourinary tract. Leiomyosarcoma (LMS) is a malignant soft tissue neoplasm which originates from the mesenchyme and has a characteristic smooth muscle differentiation. Usually, it presents as a painless, firm, slow-growing unilateral scrotal mass. Investigations include imaging, tumor markers, and histopathology. CASE PRESENTATION: A 65-year-old gentleman known diabetic and beta-thalassemic trait was referred to the Urology OPD at Letterkenny University Hospital. His presenting complaint was a left groin lump that appeared 1 year ago and was growing larger in size gradually. According to the patient, his lump was slightly painful (localized) initially that later became painless. He did not report any testicular trauma/infection or UTI. There was no significant history of malignancies running through his family. Clinical examination revealed a soft and lax abdomen, normal testes. There was a non-tender 2cm x 2cm well-circumscribed, mobile, firm to cystic irreducible left inguinoscrotal mass and appeared to be attached to the spermatic cord. Cough impulse was indiscernible. Ultrasound left groin showed 1.8 cm transverse x 1.4 cm AP x 1.9 cm sagittal) well-circumscribed ovoid nodular subcutaneous lesion present in the upper left inguinal area just lateral to the left pubic tubercle that appeared solid with heterogeneous internal echotexture and no internal calcification. Some internal vascularity is demonstrated with color Doppler assessment. CONCLUSION: Because of its rareness, LMS represents a management conundrum. There is no standard protocol for treatment. We present a case and discuss the available evidence from the literature to date to help identify LMS of the spermatic cord that is highly unusual.

Rare case of pancreatic adenocarcinoma with spermatic cord and testicular metastasis.

Male gonadal metastases from pancreatic carcinomas are extremely rare, with fewer than 20 cases documented in the literature. Herein, we report a man in his 50s who presented at the genitourinary outpatient department with an enlarged scrotum (right side) that had developed progressively over several weeks. He also reported mild upper abdominal discomfort. Scrotal sonography revealed a hydrocele on the right side without testicular lesions. A mass pancreatic tail lesion with invasion of the spleen and left adrenal gland was identified through abdominal CT and MRI. Endoscopic ultrasound fine needle biopsy and right radical orchiectomy were performed. Moderately differentiated adenocarcinoma of the pancreatic tail with hematogenous metastasis to the right testis, epididymis and spermatic cord was verified on the basis of the pathology report. Disease progression occurred despite the patient receiving palliative chemoradiation therapy.

Testicular Germ-Cell Tumors with Spermatic Cord Involvement: A Retrospective International Multi-Institutional Experience.

The 8th Edition of the American Joint Committee on Cancer (AJCC) Staging Manual designates discontinuous involvement of spermatic cord soft tissue by testicular germ cell tumors as a metastatic deposit. We conducted a retrospective international multi-institutional study to validate the current recommendations. Thirty-three (72%) nonseminomatous and 13 (28%) seminomatous testicular germ cell tumors were collected from 15 institutions in America, Europe, and Asia. Testicular tumor size ranged from 1.3 to 18.0 cm (mean: 6.1). Cases were classified as discontinuous involvement of spermatic cord soft tissue (n = 26), continuous cord involvement (n = 17), or cord lymphovascular invasion (n = 3). The mean follow-up was 39 months. Clinical stage for discontinuous involvement of spermatic cord soft-tissue patients was I (local disease) in 2/24 (8%), II (regional disease) in 6/24 (25%), and III (distant disease) in 16/24 (67%) cases; 16 (67%) patients presented with distant metastasis. Clinical stage for continuous cord involvement patients was I in 9/17 (53%), II in 4/17 (23%), and III in 4/17 (23%); 4 (23%) patients presented with distant metastasis. Disease progression was seen in 4 patients with discontinuous involvement of spermatic cord soft tissue and 5 with continuous cord-involvement (p = 0.699). When comparing discontinuous and continuous cord involvement, a significant difference was found in cord margin status (p = 0.044), spermatic cord tumor size (p = 0.016), lymph-node involvement (p = 0.037), distant metastasis (p = 0.010), individual clinical stage (p = 0.003), and nonadvanced vs. advanced disease (p = 0.003) at presentation. In multivariate analysis, after adjusting for age, histology, testicular tumor size, percent of embryonal carcinoma, lymphovascular invasion, and cord margin status, discontinuous involvement of spermatic cord soft tissue was significantly associated (p = 0.011) with advanced clinical stage at presentation. Our findings support the designation of metastatic disease for discontinuous involvement of spermatic cord soft tissue, as introduced by the 8th edition of the AJCC staging.

Several Fusion Genes Identified in a Spermatic Cord Leiomyoma With Rearrangements of Chromosome Arms 3p and 21q.

BACKGROUND/AIM: Benign smooth-muscle tumors, leiomyomas, occur in nearly every organ but are most common in the uterus. Whereas much is known about the genetics of uterine leiomyomas, little genetic information exists about leiomyomas of other organs. Here, we report and discuss the genetic findings in a para-testicular leiomyoma. MATERIALS AND METHODS: Cytogenetic, array comparative genomic hybridization (aCGH) RNA sequencing, reverse-transcription polymerase chain reaction (RT- PCR), and Sanger sequencing analyses were performed on a leiomyoma of the spermatic cord removed from a 61-year-old man. RESULTS: The karyotype was 48~50,XY,add(3) (p21),+4,+7,+8,+9,add(21)(q22)[cp9]/46,XY[2]. aCGH confirmed the trisomies and also detected multiple gains and losses from 3p and 21q. RNA sequencing detected the chimeras ARHGEF3-CACNA2D2, TRAK1-TIMP4, ITPR1- DT-NR2C2, CLASP2-IL17RD, ZNF621-LARS2, CNTN4- RHOA, and NR2C2-CFAP410. All chimeras were confirmed by RT-PCR and Sanger sequencing. CONCLUSION: Our data, together with those previously published, indicate that a group of leiomyomas may be cytogenetically characterized by aberrations of 3p and the formation of fusion genes.

Zero-ischemia testis-sparing surgery for probably benign pediatric testicular tumors.

Permanent ischemia-induced testicular damage may occur as early as 30 min in prepupertal rats. With the goal of potentially enhancing testicular function and fertility preservation, we performed testis-sparing surgery (TSS) without ischemia for testicular lesions in select children with negative markers and high likelihood of benignity on ultrasonography. Preliminary experience suggests that off-clamp TSS should be more liberally encouraged, especially in infants and prepubertal children, given their particularly vulnerable spermatic cord elements.

Para-spermatic cord proximal-type epithelioid sarcoma.

Proximal-type epithelioid sarcoma is an ultra-rare, high-grade soft tissue malignancy usually presenting as a deep-seated painless mass in the proximal extremities. Most patients are diagnosed as young adults, between 20 and 40 years of age. Perineal and genital masses do occur but are extremely rare and represent a challenging tumour to diagnose and treat. Early radical excision is recommended due to its aggressive behaviour and poor prognosis. Median overall survival from initial diagnosis is 30 months. We present the case of a 22-year-old man with a left groin proximal-type epithelioid sarcoma who is sadly deceased 12 months after initial presentation despite early surgical excision, completion of both first-line and palliative chemotherapy, and palliative radiotherapy.

Lipoblastoma-like tumor of the spermatic cord: case report and review of the literature.

Lipoblastoma-like tumor is a very rare mesenchymal tumor believed to be restricted to female patients and only recently reported in the spermatic cord of a male patient. We describe herein an additional case of lipoblastoma-like tumor occurring in the spermatic cord, describing its histopathological, immunohistochemical, and molecular features.