Diagnostic consideration of lipoma-like lesion: A case report of primary cutaneous Rosai-Dorfman disease.

INTRODUCTION AND IMPORTANCE: Rosai-Dorfman disease (RDD) is a rare disorder characterized by benign histiocytic proliferation. The purely cutaneous variant of Rosai-Dorfman disease is exceptionally uncommon. This abstract centers on an extraordinary case-an instance of primary cutaneous Rosai-Dorfman disease (PCRDD), a rare cutaneous variant within an already infrequent disorder. Successfully managed through surgical intervention, this unique case underscores the pressing need for refined diagnostic and therapeutic strategies. CASE REPORT: We present a noteworthy case of primary cutaneous Rosai-Dorfman disease (PCRDD) in a north African female, deviating from the typical age of onset. The patient exhibited a rare cutaneous variant, emphasizing the need for heightened awareness in diverse demographics. Histological analysis revealed characteristic emperipolesis and immunohistochemical features consistent with RDD. CLINICAL DISCUSSION: PCRDD, although uncommon within the Rosai-Dorfman Disease spectrum, manifests with a later onset and distinctive demographic characteristics. Surgical excision emerges as a viable therapeutic option for managing symptomatic lesions, as demonstrated in our case. The infrequency of PCRDD underscores the importance of tailored diagnostic and therapeutic strategies. Individualized approaches become pivotal in addressing the complexities associated with PCRDD, ensuring careful consideration of its intricacies in the broader context of Rosai-Dorfman disease. CONCLUSION: This case underscores the complexity of RDD, especially in rare cutaneous presentations. Surgical excision, with its reported efficacy, stands as a crucial intervention, offering both symptomatic relief and potential cure, showcasing the significance of individualized care in managing this rare disorder. Continued research remains imperative for advancing RDD understanding and refining clinical approaches.

Undifferentiated Small Round Cell Sarcoma of the Hypopharynx in a 7-Month-Old Patient.

Undifferentiated small round cell sarcoma is an extremely rare and highly aggressive tumor touching the soft tissues and bones. Here, we report the case of a 7-month-old girl who presented to our department with respiratory distress due to a hypopharyngeal mass causing airway obstruction. The patient underwent a tracheotomy and had a direct laryngoscopy. Histological examination confirmed the diagnosis of an undifferentiated small round cell sarcoma. The patient received chemotherapy and radiotherapy and underwent neck dissection. One year later, the patient was diagnosed with local recurrence as well as metastatic cervical nodes and liver and pulmonary metastases. The case is original by the tumor's location and the age of onset. To the best of our knowledge, this could be the first case in English and French literature reporting an undifferentiated small round cell sarcoma affecting the hypopharynx among infants. The purpose of this article is to report our case and discuss its clinical and anatomopathological features as well as its prognosis and treatment options.

Case Report: A very rare case of a Pleural Effusion revealing Multiple Myeloma.

Multiple myeloma is a common malignant bone-based disease. Pleural effusions reported in these patients remain rare. It is commonly due to congestive heart disease, pulmonary embolism, nephrotic syndrome or a second neoplasia. The true myelomatous pleural effusion resulting from a direct tumoral invasion of the pleural are extremely rare. We report here the case of a massive pleural effusion revealing multiple myeloma in a 71-year-old patient. The chest ultrasound showed a massive pleural effusion in the left side with a multinodular thickening of the pleura. The medical thoracoscopy showed a grape-cluster appearance. The diagnosis was made by pleural guided biopsy revealing abnormal plasma cells with an intense positive CD 138 (plasma cell marker) and MUM1 (multiple myeloma oncogene1) staining with a light kappa chain in the protein electrophoresis associated with a myeloma. Unfortunately, our patient died one month after the initial diagnosis. We present also a review of the recent literature in order to highlight the clinical presentations of the myelomatous pleural effusion, the diagnostic tools, the therapeutic strategies as well as the outcomes.

COVID-19 prophylaxis with doxycycline and zinc in health care workers: a prospective, randomized, double-blind clinical trial.

OBJECTIVES: This study aims to assess the efficacy of a combination treatment of doxycycline and zinc in the primary prevention of COVID-19 infection in Tunisian health care workers compared with two control groups. METHODS: We conducted a prospective, randomized, double-blind clinical trial over 5 months to determine the efficacy of a preventive combination treatment dose of doxycycline (100 mg/day) and zinc (15 mg/day), compared with a single-dose treatment with doxycycline versus placebo. The effectiveness of preventive treatment was measured by the significant decline in the number of cases of COVID-19 infection and/or a decrease in the viral load as determined by SARS-CoV-2 cycle threshold value using reverse transcription polymerase chain reaction tests. RESULTS: We detected a significant decrease of SARS-CoV-2 infection in the group that received both doxycycline and zinc compared with other participants. We also demonstrated that COVID-19 infection was neither associated with diabetes (P = 0.51) nor associated with hypertension (P = 0.99), asthma (P = 0.52), and chronic obstructive pulmonary disease (P = 0.27). CONCLUSION: Our findings indicated that preventive therapy reduced the risk of SARS-CoV-2. These results suggest that the combination of doxycycline and zinc has a protective effect in patients with SARS-CoV-2 infection.

Extraocular sebaceous carcinoma in a renal transplant patient: A case report.

The high risk of skin cancer after organ transplantation is a major clinical challenge. We describe a case of a patient presenting with sebaceous carcinoma (SC) after a kidney transplant. Although it is exceedingly rare, SC should always be considered in the presence of any skin lesion occurring after a transplant.

A rare case report of sacral epidural angiolipoma: Diagnosis and treatment.

BACKGROUND: Spinal angiolipomas are rare benign tumors composed of mature adipose tissue and anomalous vascular channels. The sacral localization is extremely rare. To the best of our knowledge, there have been only two cases reported in the literature. Herein, we present an additional case of sacral angiolipoma. CASE DESCRIPTION: We present a case of a 54-year-old woman who presented with the right lumbosciatica and distal crural weakness. Spinal MRI showed an epidural lesion at the level of L5-S1 extending to the first right sacral foramen. The patient had total resection of the tumor and the histological study concluded to an angiolipoma. The patients' neurologic symptoms improved postoperatively and follow-up revealed no signs of tumor recurrence. CONCLUSION: Despite the rarity of sacral localization in angiolipomas, it is a diagnosis to be considered in the case of an epidural tumor with foraminal extension. Magnetic resonance imaging is important for detecting and characterizing spinal angiolipomas despite diagnosis is not always obvious. After surgical removal, the functional prognosis is generally favorable.

Clival chordoma in a young male patient: a case report.

Chordoma is a rare malignant tumor of the spine. We report the case of a 26-year-old man who presented with facial paralysis and upper limbs paresthesia. Cerebral CT-scan and cerebro-spinal MRI revealed a 58mm locally advanced middle clival mass with deviation of median cerebral structures. Endoscopic biopsy concluded to a chondroid chordoma. Skeletal survey and thoraco-abdomino-pelvic CT-scan were normal. Treatment consisted in complete surgical removal of the tumor followed by adjuvant radiotherapy. The patient is alive free of disease with a follow up of 12 months.

Urothelial bladder carcinoma in childhood: a case report.

We report an exceptional case of transitional cell carcinoma of the bladder in a 14-years old boy without personal nor family history who consulted for a total hematuria. Work-up showed a bladder lesion sized 5cm with histology of urothelial cancer. Treatment consisted of a transurethral surgery with carcinologic complete resection. Patient is alive, free of disease with a follow-up of 36 months.

Mammary analogue secretory carcinoma: A case report.

BACKGROUND: Mammary analogue secretory carcinoma is a rare new entity of low-grade malignant tumor of salivary glands. It shared the same histologic features and the chromosomal translocation t(12;15)(p13;q25) as secretory carcinoma of the breast. AIM: To highlight the diagnosis approaches and the attitude of management in a case of MASC which is the first case reported in Tunisia. Reported case: A case of MASC of the lower left jugal mucosa was reviewed for its microscopic and immunohistochemical features. Fluorescence in situ hybridization (FISH) for the ETV6-NTRK3 translocation was performed. Surgery was the only treatment required in this case. No signs of local or regional recurrence during the one-year follow-up were noticed. COMMENTARIES: Secretory carcinoma was confused with other salivary gland tumors especially acinic cell carcinoma due to their morphological similarities, making diagnosis dilemma. Fluorescence in-situ hybridization (FISH) is the one definitive finding to confirm the diagnosis of MASC and to differentiate it from the other types of salivary gland tumor. At the present time, no specific therapy is available for patients with MASC.

Functional Bladder Paraganglioma Treated by Partial Cystectomy.

Paraganglioma is a rare neuroendocrine tumor that arises from the autonomic nervous system. The urinary bladder paraganglioma accounts for less than 0.1% of bladder tumors. It remains a challenging entity to diagnose and treat due to its rareness and the lack of specific symptoms. Treatment modalities include transurethral resection and cystectomy (partial or total). The authors report a new case of an isolated paraganglioma of the urinary bladder in a 52-year-old female patient that underwent partial cystectomy. This case aims to remind the clinical, histological and therapeutic features of this rare tumor.

A Paratesticular Angiolipoma: A Case Report of a Rare Benign Scrotal Mass and Review of Literature.

We report a rare case of paratesticular angiolipoma in a young male. The patient is a 21-year-old male who presented with a palpable firm right intrascrotal mass of 21 mm. Ultrasound findings demonstrated that it is a solid mass. Under the diagnosis of an intrascrotal solid mass, a right inguinal radical orchiectomy was performed. Histopathological examination concluded to a paratesticular angiolipoma. Angiolipoma is a rare benign form of paratesticular tumour and its diagnosis is based on histological findings of the surgical specimen with no recurrence risk. This mesenchymal tumour should be distinguished from liposarcoma, which has malignant or aggressive clinical course.

Transformation of a prostatic adenocarcinoma into squamous cell carcinoma after luteinizing hormone-releasing hormone (LHRH) agonist and radiotherapy treatment.

Squamous cell carcinoma of the prostate is rare and represents 0.5% to 1% of prostatic carcinomas. Transformation of prostatic adenocarcinoma into squamous cell carcinoma after LH-RH agonist intake has been reported in only 8 cases in the literature. To our knowledge, our case is the second pure squamous cell carcinoma observed after hormonotherapy and radiotherapy. We reported a case of a patient with prostatic adenocarcinoma treated by radical prostatectomy followed by radiotherapy. Eleven years later, he had a vesical recurrence of prostatic adenocarcinoma. Our patient had an endoscopic resection followed by injections of Triptorelin. Six months later, he developed a local recurrence of a squamous cell carcinoma.

Evaluation of concordance of Gleason score between prostate biopsy and radical prostatectomy.

BACKGROUND: Histological Gleason score grading is a main determinant of prostate cancer treatment. However, the final histological examination may reveal that concordance rates between biopsy and pathological Gleason sums are inadequate. AIM: To evaluate the concordance of Gleason score between prostate biopsy and radical prostatectomy specimen and to study factors predictive of up-grading of Gleason score at radical prostatectomy. METHODS: We conducted a descriptive and retrospective study including cases of patients who underwent  prostatectomy between 2008 and 2015. We proceeded to a histological examination of 30 cases of radical prostatectomy and 17 corresponding biopsies. The data of the remaining 13 prostate biopsies, not performed in our hospital, have been picked from detailed histological reports. RESULTS: Our results showed that the concordance in the Gleason score was 43% (kappa = 0.11, poor agreement).Gleason score was upgraded in 54% of the cases. At radical prostatectomy, it increased by two  points in one case and by one point in 14 cases. The Gleason score was under-graded on prostatic biopsies in an only 1 case. Using the new classification ISUP 2014, the concordance rate was 26% (kappa = 0.04,  very poor agreement). Gleason score was upgraded in 78% of the cases for Group 1 (SG 3 + 3) and 63% for group 2 (SG 3 + 4). The concordance rate was highest for Group 4 (4 + 3). Variables as age, serum PSA (prostate specific antigen) , numbre of cores, percentage of positive cores, or prostate volume were not significant predictors of upgrading of Gleason score on radical prostatectomy specimen. CONCLUSION: Thus, the high rate of discordance of Gleason score between prostate biopsy and radical prostatectomy specimen implies an understanding of factors predictive of discordance of this score allowing urologists, pathologists and oncologists to support patients in a more suitable way, choosing the appropriate therapeutic modality for each patient.

Paratesticular Angiomyofibroblastoma-Like Tumor: Unusual Case of a Solidocystic Form.

The angiomyofibroblastoma-like tumor of the male genital tract is a rare benign tumor. A total of 34 cases have been reported in the literature. We herein report an exceptional case of solidocystic form in its paratesticular location, in a 79-year-old man. Clinical examination objectified a right testicular induration. Macroscopic examination of the orchidectomy objectified a paratesticular solidocystic tumor formation. Microscopically, the solid area was composed of vessels with small caliber and turgidity of endothelial cells. These vessels were surrounded by clusters of epithelioid cells, sometimes having the appearance of giant cells. They were associated with spindle cells. The cystic area was uncoated. Immunohistochemically, the fusiform cells expressed Desmin and Smooth Muscle Actin. Endothelial cells and clusters of cells surrounding large vessels expressed CD34. The expression of receptors for estrogen and progesterone was negative. To our knowledge, this is the second solidocystic case of angiomyofibroblastoma-like tumor of male genital tract.