Prostatic stromal sarcoma: Report of a rare case in a young male and review of the literature.

INTRODUCTION AND IMPORTANCE: Common prostatic neoplasms are diagnosed frequently, whereas rare entities require particular awareness and special clinical management. CASE PRESENTATION: A 31-year-old man presented with dysuria, hematuria and urinary retention. Histomorphological analysis of material obtained by transurethral resection of the prostate initially favored a sarcomatoid carcinoma, but immunohistochemistry allowed the correct diagnosis of sarcoma of the specialized stroma of the prostate. CLINICAL DISCUSSION: The patient refused surgical treatment and, despite chemotherapy, he died 8 months after the diagnosis. Herein, we will highlight the diagnostic and therapeutic challenge of prostatic stromal sarcoma by illustrating this case and reviewing the relevant literature. CONCLUSION: Although rare and shadowed by more common neoplasms that may mimic it, prostatic stromal sarcoma should be considered in the differential diagnosis of bladder and prostate neoplasms because of its dismal prognosis.

Non-urothelial lesions of the urinary bladder A 14.5-year, single-institution review.

CONTEXT: In contrast to urothelial cancers, non-urothelial neoplasms involving the bladder are uncommon and often diagnostically challenging. These lesions include a variety of benign and malignant tumors often presenting with a combination of hematuria and the presence of a polypoid lesion at cystoscopy that may lead to an erroneous diagnosis of urothelial cancer. OBJECTIVE: We set out to quantify and classify the spectrum of non-urothelial lesions diagnosed in our institution, and briefly review the relevant literature on each lesion, with a focus on differential diagnosis and potential pitfalls. DESIGN: We performed a retrospective review (Jan 2008 - Jun 2022) of the cases diagnosed on TURB material at our institution. RESULTS: Out of 4071 TURB specimens, a total of 66 (1.62 %) non-urothelial lesions were identified. Most of these lesions were malignant (n = 51, 77 %), with metastases being the most common (n = 40, 60.6 %), followed by non-Hodgkin lymphoma (n = 8, 12 %). The remaining cases were benign lesions (n = 15, 22.7 %), with the most common being inflammatory myofibroblastic tumor (n = 4, 6.1 %) and endometriosis (n = 3, 4.5 %). CONCLUSIONS: In this retrospective case series, we identified various malignant and benign entities, some of which have been rarely reported in the bladder, such as paragangliomas, inflammatory myofibroblastic tumor, and leiomyosarcoma. These lesions may macroscopically and histologically mimic urothelial carcinoma. Because of their relative rarity and diagnostic overlap with conventional urothelial tumors, the pathologist should always keep in the mind the possibility of non-urothelial lesions.

Hematuria in breast cancer: don't forget bladder metastases!

The bladder is a rare site for breast cancer metastases, and only occasional reports are present in the literature. Most cases coexist with synchronous metastases elsewhere, but isolated cases of a single metastatic localization in the urinary bladder have been reported. The most common symptoms of a metastatic localization of breast cancer to the urinary bladder are hematuria and voiding dysfunction. Herein we present three cases of urinary bladder metastasis from breast carcinoma, all presenting with gross hematuria as the only symptom. After a review of the relevant literature, we discuss the clinical and histological characteristics unique to our cases, highlighting potential clinical and pathological diagnostic pitfalls and differential diagnoses.

'Burned-out' syndrome of testicular teratoma: A case report.

The majority of testicular tumors are germ cell tumors (GCTs) which, although rare, frequently present in young adults. In exceptional circumstances, spontaneous regression of the primary tumor occurs. The appellation 'burned-out' is applied to situations in which a metastatic GCT is found to be present, accompanied by histological regression of the primary testicular lesion. It is of crucial importance that a clinical examination of the testis is performed, and scrotal sonography is essential in the preliminary diagnosis of such neoplasms. In the present case report, a burned-out, non-seminomatous testicular GCT case is described. A CT scan revealed that a 29-year-old male patient who was experiencing loss of weight and appetite had retroperitoneal and mediastinal masses. A testicular examination did not reveal the presence of any palpable lesion, and an ultrasound examination of the scrotum disclosed a normal left testis and an atrophic right testicle with heterogeneous architecture, but with no evidence of a tumor. Chemotherapy was administered to the patient following surgical intervention into the retroperitoneal and mediastinal mass. It is evident that it remains problematic to accurately differentiate between a primary retroperitoneal tumor and a metastatic testicular tumor with an occult testicular primary or a 'burned-out' testicular cancer. The burned-out phenomenon is a rare occurrence, and further research into its pathogenesis is required. Both the rarity of this phenomenon and the difficulties encountered in diagnosis prompted the writing of the present case report, especially considering that teratomas are categorized as belonging to the histology group that shows the least likelihood of regressing.

Malignant melanoma of the prostate gland: A systematic review.

AIMS: Prostatic melanoma is a rare malignancy about which only scattered case reports and no systematic reviews have been published to date. We sought to better inform clinicians and pathologists caring for these patients by gathering all available evidence on the topic. METHODS: We performed a systematic review of English and non-English articles indexed in PubMed, EMBASE, Scopus and Google Scholar about primary and metastatic prostatic melanoma. RESULTS: In total, 25 studies describing 45 cases were identified. Most cases were metastases to the prostate, with only 10 primary prostatic cases. The median age of patients was 61 years with a wide range, and 89% were symptomatic at presentation, most commonly with obstructive symptoms (83%). Diagnosis requires histopathological analysis and often immunohistochemistry. Metastatic melanoma in the prostate carries a dismal prognosis with median overall survival of 3 months; on the other hand, among primary prostatic melanomas reported in the literature, 29% survived longer than 5 years. The most reasonable therapeutic approach consists in radical surgery possibly followed by adjuvant therapy.

Nervous Nipple: Pseudopolythelia Caused by a Neurofibroma of the Areola.

ABSTRACT: The presence of a supernumerary nipple inside the original areola is a rare condition termed intra-areolar polythelia. Rarely, a lesion can macroscopically resemble a nipple. We report a case of a solitary neurofibroma (by itself rare in the areola) mimicking a second, twin nipple. In this case, these 2 rare conditions merge resulting in pseudopolythelia. The relevant literature on polythelia and neurofibromas of the breast is briefly reviewed.

A Rare Case of Castleman Disease Presenting as an Ovarian Tumor.

Castleman disease (CD) is a rare lymphoproliferative disorder of unknown etiology. Its most common location is the mediastinum, but many other sites have been reported. We report a case of primary CD of the ovary, a rare localization with only 2 cases including the present case described in the world literature to date. A 58-yr-old woman who initially presented with abdominal pain underwent computed tomography scan which showed bilateral well-circumscribed solid adnexal masses. Because an ovarian bilateral tumor was suspected the patient was treated with a hysterectomy and bilateral salpingo-oophorectomy and the histopathologic examination confirmed the diagnosis of CD hyaline-vascular type of the right ovary associated with a contralateral fibroma. Three years after surgery the patient is alive and well and shows no signs of recurrent disease. The occurrence of this rare presentation of CD is the subject of this report. The problems of differential diagnosis with the most frequent lesions of the female pelvis are also discussed.

A Large Paraprostatic Mass in A Man: Rare Presentation of Aggressive Angiomyxoma.

Aggressive angiomyxoma is a rare lesion with infiltrative growth and a tendency to recur locally. We present the images of a rare case of aggressive angiomyxoma in the pelvic region of a male patient presenting as a retrovesical, paraprostatic mass causing urinary symptoms.

The first case of benign multicystic mesothelioma presenting as a splenic mass.

Multicystic mesothelioma (MM) is a relatively rare tumour arising in the pelvic peritoneum of the tuboovarian region of young woman. Exceptionally, MM occurs on the serosal surfaces of various organs including kidney, bladder, lymph nodes, and liver. We report here the first case of MM wherein a 58-year-old woman with a previous history of endometriosis of the right ovary presented with a large multicystic mass of the spleen. The diagnosis of MM was made on a surgical specimen after splenectomy. A histopathologic examination is always necessary for the diagnosis of MM which should be differentiated from other lesions particularly from cystic lymphangioma. At one year follow-up, the patient had no evidence of recurrence. Despite the high frequency of local recurrences, MM is a benign lesion and 'en bloc' surgical excision with prolonged follow-ups is the treatment of choice.

A case of multicentric low-grade neuroendocrine breast tumor with an unusual histological pattern.

Neuroendocrine features are detectable in carcinomas of the breast either as scattered cells, that are recognized by their expression of neuroendocrine cell markers. Instead, pure breast carcinomas with neuroendocrine features (NEBC) are very rare and represent <1% of all breast cancer. Usually NEBC may be well or poorly differentiated and more frequent in older woman. These tumors showed variable histological pattern but a common feature is represented by expression of neuroendocrine markers. Here we report a case of a primary multicentric low-grade neuroendocrine carcinoma of the breast presented because of its rarity and for the unusual tubular and cribriform pattern resembling a well-differentiated conventional breast carcinoma. The tumor was treated with left quadrantectomy with concomitant wide excisional biopsy of other two nodules and lymph node sentinel biopsy. No recurrence was observed during 1-year follow-up. Because of its rarity and variability of morphologic features, there exist diagnostic challenges for pathologists to differentiate primary NEBC to some conventional breast carcinomas and to the breast metastasis from neuroendocrine tumor of the lung or gastrointestinal tract. It is important to be able recognize this tumor in order to avoid potential misdiagnosis and improper management of afflicted patients.

Prognostic impact of ReTURB in high grade T1 primary bladder cancer.

PURPOSE: To evaluate whether pathological outcomes of ReTURB have a prognostic impact on recurrence and progression of primitive T1HG bladder cancer. MATERIAL AND METHODS: Patients affected by primitive T1HG TCC of bladder underwent restaging TURB (ReTURB). Patients with muscle invasive disease at ReTURB underwent radical cystectomy; those with non-muscle invasive residual (NMI-RT) and those with no residual tumour (NRT) received an intravesical BCG therapy. We compared recurrence and progression in NMIRT patients and NRT patients at restaging TURB. Patients were followed every 3-6 months with cystoscopy and urine cytology. RESULTS: 212 patients were enrolled in the study. At ReTURB, residual cancer was detected in 92 of 196 (46.9%) valuable patients: 14.3% of these were upstaged to T2. At follow up of 26.3 ± 22.8 months, there were differences in recurrence and progression rates between NRT and NMIRT patients: 26.9% and 45.3% (p < 0.001), 10.6% and 23.4% (p 0.03), respectively. Recurrence-free and progression-free survivals were significantly higher in NRT compared to NMIRT patients: 73.1% and 54.7% (p < 0.001), 89.4% and 76.6 (p 0.03), respectively. CONCLUSIONS: ReTURB allows to identify a considerable number of residual and understaged cancer. Patients with NMIRT on ReTURB have worse prognosis than those with NRT in terms of recurrence and progression free survival. These outcomes seem to suggest a prognostic impact of findings on ReTURB that could be a valid tool in management of high grade T1 TCC.

Small cell neuroendocrine carcinoma of the endometrium with pulmonary metastasis: A clinicopathologic study of a case and a brief review of the literature.

Neuroendocrine carcinomas (NEC) of the female genital tract are aggressive and rare tumors that usually involve the cervix and ovary, and are seen rarely in the endometrium in perimenopausal or postmenopausal women. We presented a case of a73 year-old postmenopausal woman with vaginal bleeding and abdominal pain. A subsequent computerized tomography (CT) scan of pelvis showed an enlarged uterus (20,0 × 12,0 cm) with para-aortic and pelvic lymph node metastases. She underwent surgical debulking and staging of an endometrial tumor with omental metastasis and positive lymph nodes. The pathological diagnosis was primary small cell carcinoma (SCC) combined with endometrioid carcinoma of uterine corpus. Her final FIGO stage was IVB. Three months after surgery CT-total body showed a metastasis to left lung of SCC. Because the small-cell component of endometrial tumor showed a strong positivity for TTF1 as pulmonary counterpart a differential diagnosis with a primary small cell carcinoma of the lung should be made. Identifying an appropriate therapeutic management for SCC of endometrium is challenging since these are extremely rare tumors. An optimal initial therapeutic approach to this rare disease, especially at an advanced stage, has not yet been clearly defined. However, in these a multidisciplinary therapy, including surgery, chemotherapy, and radiotherapy represent until this time the only therapeutic option.

Unsuspected Xp11 Translocation Renal Neoplasm Associated With Contralateral Clear Cell Carcinoma.

In this report, we present for the first time the coexistence of a conventional renal cell carcinoma (RCC) and an undefined Xp11 translocation renal neoplasm in distinct kidneys, which was difficult to definitively classify as either carcinoma or PEComa (perivascular epithelioid cell tumor). While one of the tumors showed the morphological and immunohistochemical features of clear RCC, the other had an unusual morphology with a prominent nested pattern. Microscopically this tumor showed nests of cells with clear and eosinophilic cytoplasm and nuclei with prominent nucleoli; some hyaline globules were evident. Immunohistochemical panel showed negativity for cytokeratin-pan, cytokeratin-7, PAX8, and CD10 but positive immunostaining for cathepsin K, racemase, Melan-A, and TFE3. A subsequent, metaphase, dual-color fluorescence in situ hybridization confirmed the Xp11 translocation. Attention should be paid to the routine immunohistochemical profile that, in case of negativity of specific RCC markers, may suggest an Xp11 translocation renal tumor. The addition of TFE3 can easily identify the specific subtype.

Oncocytic variant of mucoepidermoid carcinoma: a diagnostic challenge for the pathologist.

Oncocytic mucoepidermoid carcinoma of the salivary gland is rare. We report a 48-year-old man who presented with a slowly growing right parotid mass. A preoperative fine needle aspiration showed exclusively oncocytic cells and cellular debris. A first diagnosis of Warthin tumor or myoepithelioma was made. The patient underwent a superficial parotidectomy, and her postoperative course was uneventful. Histologically, the tumor was composed of solid nests and cystic gland containing clear mucoid material, lined predominantly with oncocytes and few mucous goblet cells. A final diagnosis of oncocytic variant of mucoepidermoid low-grade carcinoma was made. The recognition of this variant is important, because may be easy confused with most frequent benign tumor with oncocytic cells; in this case a complete surgical excision and long-term clinical follow-up are an adequate management.

A bone fide atypical fibroxanthoma of penis.

Malignant mesenchymal tumors of the penis are very rare and they have vascular origin. We present a case of a 71-year-old man with a painless nodule of 2.0 cm in diameter located in the penile foreskin. There was no history of urinary or sexually transmitted disease. An excisional biopsy revealed a markedly pleomorphic sarcoma resembling atypical fibroxanthoma (AFX) associated with a squamous cell carcinoma in situ. The patient refused a wide re-excision and was free of disease after 36 months. Because the different therapeutic management and prognosis, differential diagnosis should be made with sarcomatoid squamous cell carcinoma and melanoma: A diagnosis of AFX or malignant fibrous histiocytoma may be considered only after the complete exclusion of these two entities.

Axillary intranodal palisaded myofibroblastoma: report of a case associated with chronic mastitis.

Intranodal palisaded myofibroblastoma is a rare tumour of the lymph node that may be derived from myofibroblasts. The most usual area of presentation is the inguinal lymph nodes, but occurrence within other areas has also been reported. It is characterised by spindle cells, amianthoid-like fibres, and by the proliferation of hemosiderin-containing histiocytes in the lymph node. Although intranodal palisaded myofibroblastoma is benign, it is frequently confused with metastatic lesions, especially when it occurs in atypical sites. We herein report the second case of axillary intranodal palisaded myofibroblastoma occurring in a woman with a granulomatous chronic mastitis. The salient clinicopathological features of this unusual tumour are presented with emphasis to the pathogenesis of the tumour as well as to its histological and immunohistochemical characteristics. Clinicians and pathologists must be aware of this rare tumour to avoid a misdiagnosis of malignancy and assure patient a correct therapeutic management.

Glomus tumor of uncertain malignant potential on the forehead.

Malignant glomus tumors (GTs) are rare, and diagnosis requires consideration of specific histologic criteria. Glomus tumors that do not fulfill the histologic criteria for malignancy but show at least 1 feature other than nuclear atypia should be classified as GTs of uncertain malignant potential (GTUMPs). We report the case of a 74-year-old man with a slowly progressing, painful, 2.5-cm nodule on the forehead that was successfully treated via wide surgical excision and was later diagnosed as a GTUMP. Three-year follow-up showed no local recurrence or distant metastasis. Malignant GTs and GTUMPs are rare, and the nomenclature and classification of these tumors is controversial. These findings and the difficulty of differential diagnosis in a continuum between benignity and malignancy prompted our report.