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Abstract Introduction Perivascular epithelioid cell tumors are uncommon mesenchymal tumors. The genital tract is the most common extrarenal location. Preoperative diagnosis is rarely achieved owing to non-specific symptoms and imaging features. Consensus on treatment strategies remains elusive. Case presentation We report the case a 38 year-old north African woman with a primary sterility, who was diagnosed with a uterine Perivascular epithelioid cell tumor of uncertain malignant potential on a resection specimen of an intracavity polypoid mass. Immunohistochemistry confirmed the diagnosis and we opted for conservative surgery to preserve the patient’s fertility desires. Conclusion Uterine perivascular epithelioid cell tumor is a rare entity that warrants consideration in the differential diagnosis of uterine tumors. Treatment modalities, follow-up protocols, and prognosis remain ambiguous. Given their unpredictable behavior, accurate diagnosis and long-term monitoring are imperative.

Background: Inguinal lymph node (LN) metastasis and particularly the number of metastatic lymph nodes (NMLN) represent a determinant prognostic factor in vulvar squamous cell carcinoma (VSCC). However, the NMLN may be related to the number of removed LNs. Therefore, the lymph node ratio (LNR) reflects not only the burden of LN involvement but also the quality and extent of lymphadenectomy. Objectives: To investigate the value of the LNR and the count of LN on overall survival (OS) and recurrence-free survival (RFS). Design: This study is a retrospective, longitudinal, institution-based study. Methods: This study included 192 patients treated for VSCC at the Salah Azaiez Institute between 1994 and 2022. Clinical, pathological, and evolutionary data were reported. Survival curves were generated by the Kaplan–Meier method, and predictive factors of outcome were analyzed using Cox proportional hazards models. Results: Surgery consisted of a radical vulvectomy, hemivulvectomy, and pelvic exenteration in, respectively, 96.4%, 2.1%, and 1.6% of cases followed by adjuvant radiotherapy in 38.5% of cases. LN dissection was bilateral in 88.5% of cases. LNR = 0, LNR = 0–0.2, and LNR ⩾0.2 were recorded in, respectively, 64.7%, 22.1%, and 13.2% of cases. With a mean follow-up time of 35 ± 42.06 months, the 5-year OS was 52.5% and the 5-year RFS was 55.8%. On multivariate analysis, the independent prognostic factors of OS were the LNR (hazard ratio (HR) = 5.702; 95% confidence interval (CI) = 2.282–14.245; p

Abstract Introduction Vaginal metastasis from colorectal cancer is a rare occurrence, typically associated with other metastatic lesions. Isolated metastasis is exceedingly uncommon, with only a few cases documented in the literature. Vaginal involvement in colorectal cancer primarily results from direct contiguous spread from the primary tumor. Case presentation We present the case of a 70-year-old African woman diagnosed with adenocarcinoma of the middle rectum. She underwent chemotherapy, radiotherapy, and subsequent anterior resection. After 2 months, an isolated metastasis of rectal cancer was identified in the lower third of the left vaginal wall, confirmed by biopsy. Colonoscopy ruled out colorectal recurrence. Thoraco-abdominal computed tomography scan showed no distant metastases. The patient underwent abdominoperineal resection, removing the lateral and posterior vaginal wall with free macroscopic margins and a definitive colostomy. The final histopathological analysis confirmed the diagnosis of moderately differentiated adenocarcinoma of the vagina, measuring 5 × 4.5 cm. The rectal wall was extrinsically invaded by the tumor down to the muscularis propria while respecting the rectal mucosa. Resection margins were negative. The patient was discharged 1 week postoperation with no complications. Adjuvant chemotherapy was indicated, and the patient is currently tolerating the treatment well. Conclusion Vaginal metastases from colorectal cancer are extremely rare. A vigilant gynecological examination is recommended during the follow-up of colorectal cancer patients. Diagnosis can be challenging, especially if the metastatic lesion is small and asymptomatic, even after standard radiological examination. Surgical resection followed by chemotherapy is a valid option for patients with early isolated metastases.

Background and aim: As the presence of single nucleotide polymorphisms (SNPs) in the interleukin (IL)-10 gene continues to be a major challenge in the development of effective therapies for digestive cancers, this case-control study was conducted to assess the possible influence of genotype, haplotype and diplotype for two SNPs (−1082A/G (rs1800896) and −592A/C (rs1800872)) located in the promoter region of IL-10 gene on the incidence, severity and prognosis of colorectal cancer (CRC) in Tunisians. Methods: IL-10 gene SNPs were analyzed in 130 CRC cases and 165 healthy subjects (HS) using PCR-SSP. Results: For the IL-10 -1082A/G SNP, the comparison of genotype frequencies between cases and HS groups showed that the G allele significantly reduced CRC risk under the recessive model (GG vs. AA + AG: OR [95%CI] = 0.44 [0.21–0.93], p = 0.03). Conversely, a positive association was observed between the codominant model (AG vs. AA + GG) and high susceptibility (OR [95%CI] = 1.65 [1.02–2.63], p = 0.04). After stratification by disease site, the recessive model was also found to reduce susceptibility to colon cancer (OR [95%CI] = 0.18 [0.04–0.72], p = 0 0.01), while the homozygote model (AA vs. GG) was suggested as a risk factor (OR [95%CI] = 5.16 [1.31–23.26], p = 0.02). Furthermore, the codominant model (AG vs. AA + GG) doubled the risk of rectum cancer (OR [95%CI] = 1.98 [1.07–3.70], p = 0.03). For the IL-10 -592A/C SNP, the codominant model (AC vs. AA + CC) has a protective effect against the development of CRC (OR [95%CI] = 0.59 [0.36–0.94], p = 0.03). The IL-10 gene haplotype was not associated with CRC risk. A stratified analysis by disease site demonstrated that the presence of Hap3 (−1082G and −592C alleles) specifically reduced the risk of developing colon cancer (OR [95%CI] = 0.51 [0.32–0.80], p = 0.003). Moreover, homozygous Hap3/Hap3 diplotype significantly reduced susceptibility to CRC (OR [95%CI] = 0.35 [0.14–0.85], p = 0.02). Interestingly, this diplotype has not been identified in colon cancer patients. Kaplan-Meier analysis showed that the homozygous Hap2/Hap2 diplotype was significantly associated with decreased overall survival (Log-rank: p = 0.01). This association was also observed in the colon cancer subgroup (Log-rank: p = 0.001). Conclusion: Our findings provide preliminary indications that the −1082A/G and −592/AC SNPs within the IL-10 gene may exhibit significant associations with the pathogenesis and prognostic outcomes of CRC. However, further investigations are still warranted to validate and establish the veracity of our findings.

Rectal signet ring cell carcinoma represents a rare subtype of colorectal adenocarcinoma known for its aggressive biological nature and poor prognosis. Although the co-occurrence of colorectal carcinoma with other tumors has been reported, the uncommon phenomenon of tumor-to-tumor metastasis, first described in 1930, remains rare. The most frequent donor neoplasms are lung or breast carcinomas, whereas cerebral meningiomas have been reported to be the most frequent recipient neoplasms. Here we report a case of a typical lipomatous tumor harboring metastatic signet ring cell rectal carcinoma. It is about a 42-year-old man diagnosed with rectal signet ring cell carcinoma and treated with concurrent radiotherapy and chemotherapy followed by an anterior resection and manual coloanal anastomosis with a temporary ileostomy. During the surgery, an abdominal wall lipoma was discovered and excised. A histopathological examination revealed infiltration of the fibro adipose tissue by a mucinous adenocarcinoma with a contingent of signet ring cells. The patient died 12 months after adjuvant chemotherapy due to peritoneal progression. To the best of our understanding, this represents the initial documented instance of tumor-to-tumor metastasis from rectal signet cell carcinoma to a conventional nonvascular lipoma. Consequently, even if one of these tumors appears clinically and radiologically benign, it is prudent to entertain the prospect of tumor-to-tumor metastasis. Thus, a comprehensive pathologic study of both tumors is highly recommended.

Metastasis to the pancreas from malignant tumors is a rare event, representing only 1% to 2% of all pancreatic neoplasms. They occur in 2 different clinicopathological settings: as a manifestation in widespread metastatic disease or as an isolated mass in the pancreas. We report the case of a 41-year-old woman who had a history of invasive lobular breast cancer treated with radical surgery, chemotherapy, and radiotherapy. After 21 years of total remission, she presented for severe lower back pain with jaundice, nausea, and loss of 9 kg in 3 months. Abdominal computed tomography demonstrated a hyper vascularized, irregular solid lesion of 2.6 cm × 2.1 cm in the head of the pancreas with discreet biliary duct dilatation and coelio-mesenteric enlarged lymph nodes measuring 2 cm. The diagnosis of pancreatic metastasis from a lobular breast carcinoma was made by percutaneous biopsy of pancreatic lesion. The multidisciplinary committee decided a palliative treatment. The patient received chemotherapy. The take home message from his case is that we should keep in mind the hypothesis of a solitary metastasis to the pancreas, when the pancreatic lesion develops in a patient who had a clinical history of previous neoplasm especially in those which is known to potentially metastasize to pancreas.

Abstract Introduction Primary Thyroid Lymphoma (PTL) is defined as lymphoma involving the thyroid gland alone or the thyroid gland and adjacent neck lymph nodes without contiguous spread or distant metastases at the time of diagnosis. Most thyroid lymphomas are B cell lymphomas, and 98% of all PTL cases are non-Hodgkin’s lymphoma. It is a rare disease accounting for around 5% of the thyroid neoplasms and 2% of extranodal lymphomas. If properly diagnosed and treated, the prognosis is favorable. Case presentation Five cases (three men and two women) of PTL were diagnosed and treated in our institute between January 2005 and September 2019. These are 5 cases of Caucasian origin. The mean age was 76.2 (range: 63–95 years); one patient had associated hypothyroid. One patient had a medical history of breast cancer; one was hypothyroid, and four were euthyroid at the diagnosis. In 4 of these patients, PTL started with compressive symptoms. No patients underwent fine needle aspiration cytology (FNAC) or biopsy for the diagnostic only. In sonography, two cases showed bilateral nodules with goiter; in the three cases it showed nodules in the lobe and isthmus. Technetium-99m scintigraphy was performed on only two patients. Bone Marrow Biopsy (BMB) showed normal cellularity in 4 cases and only one case showed tumor cells. LDH levels were increased in all cases. The extension was evaluated in all patients with cervical and thoracic CT scans, Bone Marrow Biopsy (BMB), beta-2 microglobulin, and serum lactate dehydrogenase (LDH) levels. Three cases were staged as IE and two cases as IIE. Three patients underwent total thyroidectomy; two of them underwent cervical lymph node dissection. Two patients underwent lobectomy. All were diagnosed with lymphoma postoperatively and all were diffuse large B cell lymphoma (DLBCL). One patient completed treatment with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone), and two cases received adjuvant chemo-radiotherapy (30 Gy). Two patients died immediately after surgery. Conclusion PTL is a rare disease whose diagnosis should be considered in cases of rapidly growing goitres. Timely needle biopsy in suspected cases can avoid unnecessary surgery. Systemic treatment is required, depending on the stage of the tumour.

Abstract Background Nasopharyngeal carcinoma is known for its high potential for regional and distant metastasis. However, breast metastasis is rarely reported. Case presentation A 39-year-old Caucasian male presented with bilateral neck lymph node enlargement. Radiological examination with contrast-enhanced computed tomography scan and breast imaging revealed an enhancing mass lesion in the right breast. Histopathology of the nasopharynx mass was suggestive of undifferentiated nasopharyngeal carcinoma. A breast biopsy confirmed the diagnosis of synchronous breast metastasis from the nasopharyngeal carcinoma. We present this study to illustrate that Nasopharyngeal carcinoma can metastasize to the male breast. Furthermore, the high incidence of nasopharyngeal carcinoma metastasis underscores the pressing need to identify effective and safe strategies, emphasizing the importance of utilizing computed tomography scans for metastasis detection. Conclusion The present study illustrates the first case of synchronous male breast metastases from nasopharyngeal carcinoma. Thus, it is critical to distinguish between metastatic pathology and coexisting second malignancies to plan appropriate therapy.

Abstract Background Neurofibromatosis type 1 is a neurocutaneous genetic disorder caused by mutations in the NF1 gene, resulting in the formation of benign tumors called neurofibromas. The most common type of tumor seen in patients with neurofibromatosis type 1 is the slow-growing and benign neurofibroma, with a subtype called plexiform neurofibroma being particularly common and causing pain, functional impairment, and cosmetic disfigurement. Case presentation We report the case of a 20-year-old North African female patient with a history of neurofibromatosis type 1 who presented with a growing mass in her right gluteal region, which was later diagnosed as a giant cutaneous neurofibroma. Imaging studies revealed infiltration in several regions, including the urinary bladder wall, resulting in significant bilateral hydronephrosis. The patient is currently being monitored, and no excisional procedures are planned. Conclusions Neurofibromatosis type 1 can cause a variety of clinical symptoms, including the development of large plexiform neurofibromas. It is important to closely monitor patients with neurofibromatosis type 1 for the early detection of neurofibromas. Early detection and prompt surgical intervention are essential for preventing complications.

Abstract Introduction Cutaneous metastases (CM) revealing lung carcinoma are extremely rare, accounting for 0.8%. The diagnosis is guided by histology and immunohistochemistry. Treatment is palliative. The prognosis is poor. Case presentation This is a retrospective study of the available clinical and histological records of four North African patients with CM revealing lung cancer treated at our institute between 2004 and 2010. Three men and one woman were registered. The mean age was 54.5 years (38–74 years). Two patients had primary adenocarcinoma, one patient had small cell carcinoma and one had squamous cell carcinoma. Treatment was based on chemotherapy in two cases and antalgic radiotherapy in two cases, one patient underwent surgical resection as the lesion was infected. The overall survival after diagnosis was between one and four months. Conclusions A skin nodule can be the first symptom revealing lung cancer. A rare clinical presentation that should not be taken for a benign nodule, the biopsy and histological study with immunohistochemistry confirm the diagnosis.

Pelvic actinomycosis with an intrauterine device accounts for approximately 3% of all actinomycoses. It is a chronic infectious disease characterized by infiltrative, suppurative, or granulomatous inflammation, sinus fistula formation, and extensive fibrosis, and caused by filamentous, gram-positive, anaerobic bacteria called Actinomyces israelii . The slow and silent progression favors pseudo tumor pelvic extension and exposes the patient to acute life-threatening complications, namely colonic occlusion with hydronephrosis. Preoperative diagnosis is often difficult due to the absence of specific symptomatology and pathognomonic radiological signs simulating pelvic cancer. We discuss the case of a 67-year-old woman who complained of pelvic pain, constipation, and weight loss for 4 months, and who presented to the emergency department with a picture of colonic obstruction and a biological inflammatory syndrome. The computed tomography scan revealed a suspicious heterogeneous pelvic mass infiltrating the uterus with an intrauterine device, the sigmoid with extensive upstream colonic distension, and right hydronephrosis. The patient underwent emergency surgery with segmental colonic resection and temporary colostomy, followed by antibiotic therapy. The favorable clinical and radiological evolution under prolonged antibiotic therapy with the almost total disappearance of the pelvic pseudo tumor infiltration confirms the diagnosis of pelvic actinomycosis and thus makes it possible to avoid an extensive and mutilating surgery with important morbidity.

Objectives: Colorectal cancer (CRC) is a serious gastrointestinal disease. Cancer cells can survive in a microenvironment that includes distinct immune cells, various immune checkpoints including HLA-G, and different immune effectors such as interleukin-6 (IL-6), TNF-alpha (TNF-α), and interferon-gamma (IFN-γ). Thus, the objective of this study was to investigate HLA-G involvement in CRC as a prognostic factor in the Tunisian population, both in its soluble form (sHLA-G) and in its form linked to extracellular vesicles (HLA-GEV). Additionally, we examined its association to the secretion of cytokines. Methods: Fifty Tunisian patients, diagnosed with CRC, matched with ninety-eight healthy blood donors (HD) were enrolled in this study. Levels of sHLA-G, HLA-GEV, IL-6, TNF-α, and IFN-γ were dosed in plasma samples using specific ELISA. For the functional assay, we assessed sHLA-G effects on the production of cytokines by stimulated T lymphocytes. We measured the concentration of IL-6, TNF-α, and IFN-γ produced by activated T lymphocytes pre-stimulated by plasma sHLA-G and HLA-GEV. Results: Our case-control analysis showed high concentration of both sHLA-G (8.8 [0–63] ng/ml vs. 2.1 [0–63] ng/ml, p

Ewing’s sarcoma/primitive neuroectodermal tumor is rare and aggressive with a poor prognosis. Intraabdominal metastases are an uncommon condition. Metastasis in the lesser sac is an exceptional occurrence. To the best of our knowledge, this location has not been described previously. We report a case of a 15-year-old patient treated for Ewing’s sarcoma of the left arm 6 years back. She had developed a suspicious mass in the lesser sac 6 years following her primary tumor. The histopathologic exam revealed a tumor with “small round cells” that were positive for CD99, confirming the relapse of Ewing’s sarcoma. The relapse was successfully managed with chemotherapy and surgery. Intraabdominal, extraintestinal masses in patients treated previously for Ewing’s sarcoma should be considered as Ewing’s sarcoma relapse in the differential diagnosis. We fully describe the management of this atypical relapse, with different components of clinical, radiological, and histological findings.

Abstract Background Histologically, node-negative breast cancer generally have a good prognosis. However, 10 to 30% of the cases present local relapses or metastasis. This group of people has high chances of remission if detected early. The aim of this study is to identify financial affordability for developing countries to adjust treatment. Methods We selected 405 patients with histologically confirmed node-negative breast cancer in our institution between January 2001 and December 2003. Patients with metastasis were excluded. The statistical analysis was conducted using SPSS ver. 18 (SPSS, Inc., Chicago, Illinois). Results The medial age was 51 years old. The medial tumor size was 35.4 mm. Clinically, 67.2% of the patients were staged cT2 and 63.2%, cN1i. Breast conservation was achieved in 41% of cases. In the histologic examination, the medial size was 30 mm. Grade III tumors were found in 50.1% of patients and positive hormonal receptors in 53.4%. The mean number of lymph nodes was 14. Eight patients had neoadjuvant chemotherapy. Adjuvant locoregional radiation and adjuvant chemotherapy were prescribed respectively in 70.6 and 64.4% of cases. 59.7% had adjuvant hormonal therapy. The follow-up showed 17.7% cases of relapse either locally or in a metastatic way in a mean time of 57.4 months. The disease-free survival at 5 years was 82.1%, and the overall survival for the same period was 91.5%. The histologic tumor size and the grade and number of lymph node dissected were shown to be influencing the disease-free survival. Radiation therapy and hormone therapy showed improved disease-free survival and overall survival. Conclusion Our study found interesting results that may help personalize the treatment especially for patient living in underdeveloped countries, but further studies are needed to evaluate those and more accessible prognostic factors for a more accessible healthcare.

Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma of soft tissue representing about 1% of all tumors. In addition, DFSP occurs commonly on the trunk and extremities, and only a few cases of DFSP have been observed on the breast. In men, only 11 cases, including this case, have been reported. In this article, we present a case of left breast DFSP that occurred in a 44-year-old man. The physical examination revealed a left breast tender mass, which invaded the skin. The tumor was staged as T4b N0 M0. Mammography and sonography showed a suspect mass of the left breast. The biopsy and immunochemistry permitted the diagnosis of DFSP of the left breast. The patient had a left mammectomy, with free margins. He presents no evident sign of recurrence 7 months later.

Adenoid cystic carcinoma is an uncommon malignant neoplasm of the salivary gland. Liver metastasis from salivary gland cancer is a rare situation. In this article, we report the case of a 29-year-old woman treated 5 years previously for adenoid cystic carcinoma of the parotid gland by surgery and radiotherapy, who presented for a large hypervascularized hepatic metastasis of 20 cm. After 3-cycle chemotherapy stability, hepatic surgery was successfully performed. The patient maintained disease-free period of 12 months after the surgical treatment. This rare case represents a therapeutic challenge for oncologists and surgeons. Through this case and a review of the literature, we try to better detail the management of this uncommon entity.

Fibroadenomas are the most common breast disease that occurs usually in young. The coexistence of an invasive ductal carcinoma and a fibroadenoma in the ipsilateral breast is extremely rare. We present the case of a 52 years woman, presented to us for an upper-outer breast lump. Breast imaging concluded to tow contiguous lesions, one of them was suspicious. She had a conservative surgery. Histology concluded to a fibroadenoma and an invasive ductal carcinoma.

Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin that rarely presents in the vulva, typically occurring on the trunk of young to middle-aged adults. Even though it has a low potential for distant metastases, it often recurs locally. Therefore, surgical excision is the treatment of choice. We report a case of DFSP with fibrosarcoma transformation involving the vulva treated by large excision with tumor free margins followed by plastic reconstruction. Our aim is to highlight this rare disease and through literature evaluate the safety and efficacy of surgical treatment. Early recognition of this rare entity whose localization and the aggressive nature of the fibrosarcomatous component will be an issue in the surgical management.

Background: Molecular classification of breast tumors has identified the basal-like subtype, with high heterogeneity and very poor prognosis. These tumors are mainly triple negative, characterized by the expression of basal markers CK5/6 and EGFR. In this study, we sought to investigate the features, outcome, and therapeutic modalities of basal-like breast cancers (BLBC). Methods: We retrospectively identified 90 BLBC patients diagnosed at the Department of Surgical Oncology of Salah Azaiez Institute between January 2009 and December 2013. Results: The mean age of our patients was 50 years, and 15.5% had a family history of breast cancer. The mean tumor size was 43.8 mm. Histological examination revealed invasive ductal carcinoma in 88.9% of the cases, metaplastic carcinoma in 5.6%, and medullary carcinoma and adenoid cystic carcinoma in 2.2%. BLBC was most often associated with a high tumor grade (55.3% had a grade 3 tumor) and a high Ki-67 proliferative index. Vascular invasion was found in 31.1% of the cases. Regarding lymph node involvement, 42.9% had positive lymph nodes and 7.9% featured distant metastases. Surgical treatment was provided for 85 patients. It consisted of conservative surgery in 40 cases and radical surgery in 45 cases. Neoadjuvant chemotherapy was administrated to 23 patients, with a 13% complete pathologic response. The rates of overall survival and disease-free survival at 3 years for localized BLBC were 74.4% and 75.9%, respectively. Conclusion: BLBCs are aggressive tumors associated with poor prognosis. Thus, to identify novel prognostic factors and therapeutic targets, prospective studies should investigate the epidemiological and evolutive profile of these tumors.