High-dose-rate brachytherapy in scrotal extramammary Paget's disease: A case report.

PURPOSE: Extramammary Paget's disease (EMPD) is a rare but lethal intraepithelial malignancy without standardized guidelines concerning diagnostic or therapeutic approaches. We report a case of EMPD of the scrotum treated with excellent results using high-dose brachytherapy. METHODS AND MATERIALS: A 76-year-old male originally presented in 2015 with pruritus and erythema of the right scrotum, biopsy proved to represent extramammary Paget's disease. He was treated for a year with topical creams without sustained relief. In July 2016 he underwent a right hemiscrotectomy which revealed stage 1 EMPD of the right scrotum and the medial thigh with positive margins but no deep invasion. Brachytherapy was selected as the most appropriate treatment option and carried out in December 2016 using HDR with a H.A.M. applicator and CT treatment planning. RESULTS: On December 2021, at 5 years of clinical and pathological follow up, the patient remains NED with minimal skin toxicity. CONCLUSIONS: High-Dose-Rate Brachytherapy appears to be a feasible treatment alternative as adjuvant therapy in patients with EMPD with incomplete resection.

Adjuvant radiotherapy in the treatment of dedifferentiated liposarcoma of the spermatic cord: a rare entity.

Dedifferentiated liposarcoma (DDL) of the spermatic cord is a very rare entity in which management is remarkably controversial due to scarce literature. Although the actual standard of care is surgery via radical inguinal orchiectomy, adjuvant therapies like radiotherapy have demonstrated improved local control, particularly in cases with higher risk for local recurrence and worse prognosis. The role of adjuvant chemotherapy remains questionable in this subset of patients. On literature review, the most important prognostic factors for survival outcomes were surgical margin status, histological grade and the presence of metastases prior to the initial surgery. In this report, we discuss the case of a 59-year-old man with DDL of the spermatic cord that was treated with surgery followed by adjuvant radiotherapy. We also provide a comprehensive literature review about the management of this entity.

Safety and efficacy of intensity-modulated radiotherapy in the management of spermatic cord sarcoma.

PURPOSE: Spermatic cord sarcoma is a rare disease, which management remains controversial due to the lack of guidelines. The standard therapeutic approach is surgical: wide soft-tissue resection with radical inguinal orchidectomy, The diagnosis is made during the analysis of the specimen. The high rate of local recurrence indicates adjuvant radiotherapy of the tumor bed. The aim of this series is to determine the efficacy and safety of postoperative intensity-modulated radiotherapy for spermatic cord sarcomas. PATIENTS AND METHODS: Our series included five consecutive cases of spermatic cord sarcoma treated between 2011 and 2014. The indications for radiotherapy were: R1 status after initial surgery, R1 status after wide en bloc resection and orchiectomy, high French federation of cancer centers (FNCLCC) grade, tumor size over 5cm, tumor resection during surgery. RESULTS: Median age at diagnosis was 66years (range 46-84years). Median follow-up was 18months (range 6-28months). Four patients had repeat surgery after incomplete removal. All surgeries were orchidectomy with primary ligation of testicular vessels. One patient did not have an in sano margin after the second surgical procedure. The median tumor size was 60mm (range 30-150mm). No recurrence was observed during the follow-up. CONCLUSION: No grade 4 toxicities were reported and the most frequent acute toxicity was dermatitis. No recurrence was reported after adjuvant intensity-modulated radiotherapy. The treatment is feasible and well tolerated and seems to provide encouraging results regarding locoregional control of the disease. Dynamic or rotational intensity-modulated radiotherapy is now recommended to decrease acute toxicities while improving the efficacy of this approach.

Diagnosis and management of spermatic cord tumors.

PURPOSE OF REVIEW: Spermatic cord tumors (SCT) are very rare. The present review discusses the most recent literature regarding clinical presentation, pathological characteristics, diagnosis, and management of SCT. RECENT FINDINGS: Although the majority of SCT are benign, when malignant almost all SCT are sarcomas. Liposarcomas are the most common; whereas rhabdomyosarcomas recorded the highest tendency of develop distant metastases. The clinical presentation is usually a unilateral solid slow-growing mass at the level of the inguinal canal and of the scrotum. Surgical excision represents the most common used treatment, and considering the risk in developing local recurrence, radical inguinal orchiectomy and resection of the tumor with negative microscopic surgical margins is mandatory. Adjuvant therapies such as radiotherapy and chemotherapy have been suggested in selected patients, but clear data to demonstrate any improvement in survival are not available. SUMMARY: SCT are rare tumors with high risk of misdiagnosis or mistreatment. The majority are benign, but when malignant almost all are sarcomas. A surgical excision is the treatment of choice; however, no clear data exists documenting the efficacy of a multimodal treatment in reducing high local recurrence rates after surgery.

Three-dimensional surface imaging for detection of intra-fraction setup variations during radiotherapy of pelvic tumors.

PURPOSE: Surface-based image guided radiotherapy (IGRT) allows positioning and/or monitoring patients in 3 dimensions (3D), without the use of ionizing radiation. In this study, we report on intra-fraction motion measured by acquisition of multiple images of 3D body surfaces. MATERIALS AND METHODS: Twenty-nine patients treated for pelvic tumors were enrolled. Setup variations (SV) through three consecutive body surfaces acquired by the optical IGRT system Align-RT (Vision-RT, London, UK) were analyzed before, during and at the end of treatment delivery. Displacements along the main axes (X, Y and Z) from initial (I) to mid-treatment (MT) and final (F) acquisitions were recorded. Time and direction of SV were assessed. RESULTS: A total of 6272 images from 792 fractions of 29 patients were available. The main source of misalignment was between I and MT acquisition (p < 0.001). The dominant SV direction was the vertical one (Z axis), with mean SV of -1.20 ± 0.06 mm and -1.55 ± 0.06 mm for I-MT and I-F acquisitions, respectively. The Y mean components of SV were, respectively, -0.95 ± 0.10 mm and -1.0 ± 0.10 for I-MT and I-F acquisitions, while the X deviations were 0.07 ± 0.08 mm for I-MT and 0.26 ± 0.08 mm I-F. CONCLUSION: Three-D surface imaging for patient setup monitoring highlighted remarkable mobility of patients during RT session, especially in the anterior-posterior direction (Z axis). The largest magnitude in patient movements occurred during the first part of delivery. These findings suggest that the initial setup control cannot not to be sufficient to guarantee treatment reproducibility, especially for long-lasting RT treatments.

Pulsed dye laser versus long pulsed Nd:YAG laser in the treatment of angiokeratoma of Fordyce: A randomized, comparative, observer-blinded study.

BACKGROUND: Angiokeratoma of Fordyce is typically asymptomatic, blue-to-red papules with a scaly surface located on the scrotum, shaft of penis or labia majora. They can be treated with some locally destructive treatment modalities such as excision, electrocoagulation, cryotherapy and laser. OBJECTIVES: To compare the effects of the pulsed dye laser versus long pulsed Nd:YAG laser in the treatment of angiokeratoma of Fordyce. METHODS: Twenty tow patients with angiokeratoma of Fordyce were included in this study. All participants received three sessions of pulsed dye laser on the selected side or part of lesional area and long pulsed Nd:YAG laser on the other side or part of lesional area. Two dermatologists independently evaluated the photographs of the baseline and two-month follow-up after last session using a grade system in which treatment response was categorized into six grades. RESULTS: Both PDL and long pulsed Nd:YAG laser revealed statistically significant improvements in angiokeratoma of fordyce. Comparatively, there was a statistical difference between them (overall mean improvement with PDL, 61.8%, versus Nd:YAG, 77.63%; p < 0.005). CONCLUSIONS: Both PDL and 1064-nm Nd:YAG laser are effective and safe in the treatment of angiokeratoma of Fordyce with better response in Nd:YAG laser than pulsed dye laser.

Angiokeratoma of Fordyce response to long pulsed Nd:YAG laser treatment.

Angiokeratoma of Fordyce can be easily diagnosed by their typical erythra and cured by varied therapeutic methods including surgery, electrocoagulation, cryotherapy, or various laser systems. Which are the optimal therapeutic methods? There are no consentaneous opinions in the reported articles. We present our characteristic treatment with the application of long pulsed neodymium-dopedyttrium-aluminum-garne (lpNd:YAG) laser in the treatment of angiokeratoma of Fordyce. A 1064 nm lpNd:YAG laser (spot size: 5 mm/7 mm, energy:90-130 J/cm(2) , and pulse duration: 10-20 ms.) was used to treat the patient's lesions. The desirable clinical endpoint of the treatment was lesions shrunk and turned pallor immediately after the irradiation. The treatment interval was at least 8 weeks. Of the 11 patients, 9 of them were cured and 2 of them were improved. The mean treatment sessions were 2.2 times. None of them had a scar formation and any other side effects. All of them were satisfied with the treatment results. We conclude that angiokeratoma of Fordyce responded well to lpNd:YAG laser treatment. It provided a simple, rapid, and no bleeding treatment in treating Angiokeratoma of Fordyce.

External beam radiotherapy of extramammary Paget disease.

Extramammary Paget disease (EMPD) is an insidious intraepithelial neoplasm that is difficult to control with surgery, as large resections typically are required. An effective alternative is external beam radiotherapy (EBRT), which typically results in rapid resolution of EMPD. In this study, we analyzed long-term outcomes in 7 patients who were treated with EBRT for EMPD.

Postoperative radiation therapy for extramammary Paget's disease.

BACKGROUND: Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy that is usually treated with surgery. Patients with positive surgical margins require adjuvant therapy, but there have been few reports on the use of radiation therapy. OBJECTIVES: To investigate the effectiveness of postoperative radiation therapy in EMPD. MATERIALS AND METHODS: Twenty-one patients with EMPD involving the genitalia underwent radiation therapy as adjuvant therapy after surgery. Ten patients had inguinal lymph node involvement before radiation therapy, but none had distant metastases. A median total dose of 59·4 Gy (range, 45-64·8 Gy) was delivered to the tumour bed in 30 fractions (range, 23-36 fractions). RESULTS: At a median follow-up period of 38 months, all patients had local control. However, six patients had developed distant metastases 6-43 months after radiation therapy. The distant metastasis-free rates were 66% at 3 years and 55% at 5 years. Inguinal lymph node involvement was a significant risk factor for distant metastases. Four patients died 33-58 months after irradiation; the causes of death were tumour progression in three patients and infectious pneumonia in one. The overall and cause-specific survival rates were both 92% at 3 years, and 62% and 71% at 5 years, respectively. No therapy-related toxicities of grade ≥ 3 were observed. CONCLUSIONS: Postoperative radiation therapy is safe and effective in maintaining local control in patients with EMPD.

Extramammary Paget's disease: analysis of 17 Chinese cases.

BACKGROUND/OBJECTIVE: Extramammary Paget's Disease (EMPD) seems to be more common in Caucasians than Chinese. We report the clinical manifestations, management, and prognostic characteristics in 17 Chinese patients. METHODS: Medical records and biopsies of 17 patients who had been treated at a large university hospital in China between March 2005 and January 2012 were reviewed. RESULTS: Of the 17 patients, 14 were men. They had lesions on the scrotum and the penis. Of the three women, two had vulvar and one had inguinal lesions. All patients underwent Mohs micrographic surgery (MMS). Three men had metastasis to the inguinal lymph nodes and underwent an extensive local excision with inguinal lymphadenectomy. Eight patients who had positive excision margins received additional radiation therapy. The mean follow-up duration was 54 months (4-85 months). One patient had two recurrences. Three had metastasis to the inguinal lymph node. One had metastasis to the bone and concomitant prostate cancer. Two patients died of the disease. CONCLUSION: A striking difference in presentation of EMPD in Chinese compared with Caucasians is the male predominance and location on the penis and scrotum. Mohs micrographic surgery followed by radiotherapy is an effective treatment. Long-term follow-up suggests that the disease has a good prognosis when it does not metastasise.

Paratesticular liposarcoma: unusual patterns of recurrence and importance of margins.

BACKGROUND: Paratesticular liposarcoma (LPS) is a rare entity for which optimal treatment has not been defined. We sought to determine recurrence patterns and prognostic factors. METHODS: A total of 25 patients with localized paratesticular LPS between 1987 and 2009 were reviewed. Actuarial local-recurrence-free survival (LRFS), disease-free-survival (DFS), and overall survival (OS) were determined using the Kaplan-Meier method. RESULTS: LPS histology was well differentiated for 10 patients (40 %), de-differentiated for 14 (56 %), and pleomorphic for 1 (4 %). Final margins were positive in 8 patients (32 %). Radiation therapy (RT) was given to 10 patients; fields included inguinal canal ± scrotum and low pelvis. LRFS rates at 3 and 5 years were 76 and 67 %. The 3-year LRFS rates were lower in patients with positive margins compared with those with negative margins (29 vs 100 %, p = .0005) and in patients with recurrent versus primary disease (38 vs 83 %, p = .04). Among patients who received surgery and RT, margins remained a significant predictor of local recurrence (p = .009). Interestingly, recurrences in 4 patients tracked along gonadal vessels, and only 1 patient had a distant recurrence. OS at 5 years was 100 %. CONCLUSIONS: For patients with localized paratesticular LPS, positive margins and presentation with recurrent disease are adverse prognostic factors for LRFS. LR for patients with positive margins is still high despite RT; thus aggressive surgery to attain negative margins should be attempted in all cases. The finding of regional recurrences along gonadal vessels should be validated, and imaging studies should be tailored to reflect potential patterns of disease at presentation and subsequent recurrence.

Radiotherapy for spermatic cord sarcoma.

OBJECTIVES: Spermatic cord sarcomas are rare paratesticular tumors affecting older men. Current management is based on small series, case reports, and literature reviews, with surgery still the mainstay of treatment. Local-regional recurrence is common after definitive surgery (~50%), but patients treated with adjuvant radiotherapy may have improved outcomes. METHODS: We reviewed the outcomes of 15 patients with intermediate-grade to high-grade spermatic cord sarcomas treated with radiation at our institution from 1974 to 2009. Patients were treated to 40 to 60 Gy using conformal opposed anterior-posterior/posterior-anterior ports to the scrotum, inguinal canal, and lower pelvic wall with various beam energies. Some patients were managed with surgical exploration and resection, followed by radiotherapy and/or definitive surgery. More recently treated patients had an initial biopsy, followed by preoperative radiation or planned resection with postoperative radiation therapy. RESULTS: No patient experienced a local recurrence. Two patients had regional nodal recurrences and 1 had distant metastases. All recurrences were in patients who had initial "exploration" with unexpected findings of sarcoma during surgery versus planned, definitive resection with planned adjuvant radiotherapy. At 5 years, overall survival was 53%, but cause-specific survival was 80%. Complications were minimal, with only 4 grade 2 or 3 toxicities and no grade 4 toxicities. CONCLUSIONS: Although most patients die from causes other than disease progression, this sarcoma carries grave morbidity. Optimizing the primary management is of utmost importance. Unplanned treatments complicate definitive therapy and increase the risk of local-regional contamination and recurrence. Proactive management is therefore consistent with sarcomas of other primary sites, ideally with preoperative radiotherapy and definitive resection.

[Radiation therapy in locally advanced and/or relapsed urological tumors]. / Ruolo della radioterapia nei tumori urologici localmente avanzati e/o recidivati.

Radiation therapy (RT) plays a fundamental role in the treatment of locally advanced and/or relapsed urological tumors, as well as in palliation, or as definitive treatment, and even where integrated into a multi-modal approach. In operated renal tumors, positive margins or extracapsular extension show a positive impact of postoperative RT, with a reduction of relapses between 100% and 30%, while, in the case of palliation, treatments with RT at high doses are preferred. In advanced cancers of the upper urinary tract, RT plays a limited role, even if it seems to increase the level of disease control locally and, with the combination of cisplatin, survival rates too. An important reduction in the recurrence is also observed in locally advanced tumors of the urethra, with a recurrence of 60% after surgery, 36% after RT and 25% after pairing of the two. In locally advanced tumors of the penis, RT shows poorer results than surgery, and the addition of postoperative RT does not seem to add any further outcome, except where, in the presence of a positive inguinal dissection, the postoperative RT reduces lymph node recurrences by 60%-11%. Interesting data for the preservation of the organ are reported with reference to the combination with chemotherapy. In the tumors of the testis, it is still disputable whether the treatment of residual masses after chemotherapy may be appropriate, with a view to a possible salvage radiotherapy. In the treatment of the prostate, the role of RT is consolidated and evolving with the progress of dose escalation, the association with hormonal therapy, new technologies, new possibilities of IMRT and proton therapy and various studies on multi-modal approaches (hormone therapy, surgery, radiotherapy, chemotherapy). Cystectomy is the gold standard for the treatment of locally advanced bladder cancer, even though there is a revived interest in multimodal treatments (transurethral resection, chemotherapy, RT) that may allow the organ preservation. Postoperative radiotherapy, which can reduce by 50% to 20%-5% local recurrences that are highly correlated with distance failure and with survival, should be revised in the light of modern RT techniques that can further increase local control levels and reduce the toxicity significantly.

Single-arc volumetric-modulated arc therapy can provide dose distributions equivalent to fixed-beam intensity-modulated radiation therapy for prostatic irradiation with seminal vesicle and/or lymph node involvement.

OBJECTIVES: Volumetric-modulated arc therapy (VMAT) is becoming an increasingly utilised modality for treating a variety of anatomical sites. However, the efficacy of single-arc VMAT to treat prostate cancer suspicious for extraprostatic extension was heretofore unknown. In this work, we report our institutional experience with single-arc VMAT and fixed-beam intensity-modulated radiation therapy (IMRT) for prostate cancer patients treated for seminal vesicle and/or lymph node involvement. METHODS: Single-arc VMAT and 7- or 9-field IMRT treatment plans were compared for 10 prostate cancer patients treated for seminal vesicle involvement and/or lymph node involvement. All treatment plans were constructed using the Philips Pinnacle treatment planning system (v.9.0, Fitchburg, WI) and delivered on an Elekta Infinity radiotherapy accelerator (Crawley, UK). Resulting plans were compared using metrics that characterised dosimetry and delivery efficiency. RESULTS: No statistically significant differences in target coverage, target homogeneity or normal tissue doses were noted between the plans (p>0.05). For prostate patients treated for seminal vesicle involvement, VMAT plans were delivered in 1.4±0.1 min (vs 9.5±2.4 min for fixed-beam IMRT) (p<0.01) and required approximately 20% fewer monitor units (p=0.01). For prostate patients treated for lymph node involvement, VMAT plans were delivered in 1.4±0.1 min (vs 11.7±1.3 min for fixed-beam IMRT) (p<0.01) and required approximately 45% fewer monitor units (p<0.01). CONCLUSION: Single-arc VMAT plans were dosimetrically equivalent to fixed-beam IMRT plans with significantly improved delivery efficiency.

Role of radiotherapy as curative treatment of extramammary Paget's disease.

PURPOSE: Extramammary Paget's disease (EMPD) is a relatively rare malignancy that usually arises in the genital areas. Wide surgical excision remains the standard and most reliable curative treatment of EMPD. However, surgery is sometimes not possible, because many patients are elderly, and complete excision can be difficult owing to the tumor location. We, therefore, performed a review to determine the role of radiotherapy (RT) for EMPD. METHODS AND MATERIALS: A total of 22 patients with EMPD in their external genitalia (4 men and 18 women, age 52-94 years at RT) underwent RT with curative intent. Nine patients had regional lymph node metastases. A total dose of 45-70.2 Gy (median, 60) was delivered to the pelvis, including the tumors, in 25-39 fractions (median, 33). RESULTS: In all but 3 patients, the irradiated tumors were controlled during a follow-up period of 8-133 months (median, 42). Of the 22 patients, 13 developed recurrences, including local progression within the radiation field in 3 and lymph node and/or distant metastases outside the radiation field in 10, at 3-43 months after treatment. The 2- and 5-year local progression-free rates were 91% and 84%, respectively. Of the 22 patients, 7 patients had died at 33-73 months after RT. The cause of death was tumor progression in 4, infectious pneumonia in 2, and renal failure in 1 patient. The overall and cause-specific survival rates were 100% for both at 2 years and 53% and 73% at 5 years, respectively. No therapy-related Grade 3 or greater toxicity was observed. CONCLUSIONS: RT is safe and effective for patients with EMPD. It appears to contribute to prolonged survival as a result of good tumor control.

Role of radiotherapy in the treatment of fibrosarcoma of the spermatic cord: a case report and review of the literature.

BACKGROUND: Spermatic cord sarcomas are rare. The therapeutic approach is based only on case reports and small series. The standard treatment is radical orchiectomy with wide local resection, while the role of adjuvant therapies is not clear. We present a case of fibrosarcoma of the spermatic cord treated with surgery and adjuvant radiotherapy. A review of the literature about the role of adjuvant treatments is also discussed. CASE REPORT: A 59-year-old man presented a right testicular mass of about 4 × 3 cm in size. Biopsy showed a high-grade polymorphous sarcoma, consistent with a diagnosis of poorly differentiated fibromyosarcoma. He underwent a right radical inguinal orchiectomy and adjuvant radiotherapy (total dose: 5940 cGy). During treatment the patient developed a G3 skin toxicity (RTOG score) in the inguinal fold. After a follow-up of 57 months, he is alive and without evidence of local or distant recurrence. No late toxicity was noted. CONCLUSION: The optimal adjuvant management of spermatic cord sarcoma is still uncertain. Looking at the literature, it seems that adjuvant radiotherapy can improve locoregional control and disease-free survival without additional late toxicity.

Long-term outcomes and patient satisfaction of continent catheterizable limb and augmentation cystoplasty simultaneous with salvage prostatectomy.

AIMS: We evaluated the long-term outcomes of augmentation cystoplasty and continent catheterizable limb formation in patients who underwent simultaneous salvage prostatectomy. METHODS: Twelve men who underwent salvage prostatectomy with augmentation cystoplasty and either an appendicovesicostomy or Monti ileovesicostomy between October 2000 and February 2003 were assessed for long-term surgical complications, reoperations, continence rates, and patient satisfaction using the Incontinence Symptom Index (ISI) questionnaire and a self-designed catheterization questionnaire. RESULTS: In addition to bladder augmentation, six patients underwent appendicovesicostomy, four had a Monti ileovesicostomy and two a spiral Monti ileovesicostomy. There were no intraoperative complications or surgical-related deaths. Four patients required revision surgery (33%) at a mean of 39 months. Minor revisions were performed for stomal stenosis in two patients with stomal relocation in two patients. Ten of 12 patients were dry (83.3%) with a catheterization frequency of between 3 and 8 hr. At a mean follow-up of 61 months (SD = 20.51), the mean ISI severity score was 1.86 (SD = 3.54) and the mean ISI bother score was 1 (SD 0.74). The majority (86%) would choose to undergo the surgery again. CONCLUSIONS: Although surgically challenging and associated with significant morbidity, salvage prostatectomy with concomitant bladder augmentation and continent catheterizable reconstruction is a feasible and effective means of obtaining adequate long-term urinary continence, while preserving the native bladder. In comparison to similar historical patients with catheterizable limbs only, fewer of the augmented patients needed antimuscarinic medication and delayed augmentation was not necessary. Complications do not increase over time and the continence rates are stable.

[Spontaneous rupture of the femoral artery after radiotherapy: a case report]. / Rottura spontanea dell'arteria femorale dopo radioterapia: presentazione di un caso clinico.

Radiation-induced arteriopathy is a well-known disease whose incidence is not known and which usually arises chronically many years after radiation therapy. When it arises acutely, spontaneous rupture or, more rarely, thrombosis of the involved vessel may occur. Spontaneous rupture can occur within 4 to 32 weeks of radiotherapy, and usually affects the carotid artery involved in radiotherapy of the neck and head. Spontaneous rupture of the femoral artery is a very rare event and only a few cases have been reported in the literature. In this paper we report a case of spontaneous rupture of the left femoral superficial artery after adjuvant radiotherapy following surgery for a liposarcoma of the spermatic cord with multiple local recurrences, successfully treated with an extra-anatomic bypass through the obturator canal and rectal muscle flap.