Intraabdominal and ganglionic desmoplastic small round cell tumor: a case series.

INTRODUCTION: Desmoplastic small round cell tumor is a rare malignancy with poor prognosis, affecting young male patients. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. In late stages, metastases may be present. AIM: We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of four patients with desmoplastic small round cell tumor at our institution. CASES PRESENTATION: The first three cases reported are 32-, 17-, and 30-year-old North African males with intraabdominal desmoplastic small round cell tumor treated by surgery, chemotherapy, and radiation therapy with different follow-ups. The final case is a 16-year-old North African male with ganglionic desmoplastic small round cell tumor but no evidence of a tissue mass. He underwent two lines of chemotherapy with no response. The patient was lost after 2 years of follow-up. In all cases, desmoplastic small round cell tumor was confirmed by presence of t(11,22) (p13,q12) translocation. CONCLUSION: Treatment of desmoplastic small round cell tumor is based on multidisciplinary therapy. Despite high-dose chemotherapy, extensive surgical resection, and radiotherapy, desmoplastic small round cell tumor remains lethal.

Uterine cervix metastasis from primary colon adenocarcinoma: a case report and review of the literature.

INTRODUCTION: Metastases to the female genital tract from extragenital primary tumors are unusual. We report a rare case of uterine cervix metastasis from colon adenocarcinoma and discuss diagnostic and therapeutic issues. CASE REPORT: We report a case of a 38-year-old North African Caucasian woman treated for a non-metastatic colon adenocarcinoma. She had a sigmoidectomy and incomplete adjuvant chemotherapy. Six months later, she consulted with vaginal bleeding caused by a cervical tumor, which was confirmed to be metastatic disease, and the patient underwent decompressive and hemostatic radiotherapy. CONCLUSION: Uterine cervix metastasis from primary colon adenocarcinoma is rare. The resection remains the standard protocol for the local treatment of resectable metastatic disease. Otherwise, systemic therapy is the preferable option.

Primary ectopic axillary breast cancer: a case series.

INTRODUCTION: Ectopic breast tissue is present in 2-6% of women. Ectopic breast cancer represents an uncommon disease accounting for about 0.3% of all breast neoplasms, limiting the available evidence. Thus, we aim to report long-term outcomes in five cases treated at our institution. CASE SERIES: Our Tunisian patients' median age was 48 years (33-60 years), and the median follow-up was 8 years (4-10 years). The ectopic breast tissue was located four times in the right axilla. The median tumor size was 25 mm (15-55 mm). Four of the patients underwent a wide local excision and axillary lymph node dissection. Three of those women had positive lymph nodes; thus, they received adjuvant chemotherapy, radiation therapy, and hormone therapy. The patient with a negative lymph node (case 5) had adjuvant radiation therapy and hormonal therapy. One of the patients (case 1) had a positive supraclavicular lymph node and received radiation therapy, chemotherapy, and hormonal therapy. The latter developed a locoregional relapse after 4 years and was treated with mastectomy and chemotherapy. One patient (case 4) had a distant metastasis after 2 years of follow-up and received chemotherapy. The three other patients were free of relapse during their follow-up period. CONCLUSION: Primary axillary breast carcinoma is a rare entity. Despite the paucity of literature, our findings and authors' recommendations suggest that local excision can be performed safely with promising outcomes in this subset of patients.

Giant Darier-Ferrand dermatofibrosarcoma protuberans of the abdomen and pelvis: a case report.

BACKGROUND: Darier-Ferrand dermatofibrosarcoma (DFS) is a rare malignant cutaneous neoplasm characterized by local aggressiveness, high risk of recurrence, and low metastatic potential. CASE PRESENTATION: A 60-year-old Tunisian man with recurrent abdominopelvic DFS for which he had undergone multiple excisions presented with an extensive DFS that invaded the external genitals. He underwent a large excision with emasculation and thin cutaneous graft of the abdominal wall and local skin flap in the pelvis. CONCLUSION: DFS is a rare yet recurrent skin tumor. Wide excision with free margins remains the cornerstone of treatment. We report a case of a giant DFS treated with wide excision and reconstructive surgery to cover the defect.

Breast tuberculosis: a case series.

BACKGROUND AND AIM: Breast tuberculosis is a rare disease, even in endemic areas. The diagnosis can be challenging, as it can mimic breast cancer. We aim to report our experience and discuss diagnoses and management modalities. RESULTS: We encountered twelve cases of breast tuberculosis in our institution from 2004 to 2019. The average age of our Caucasian North African patients was 42 years old (22-63). The classic presentation was a breast lump found in half of the cases. On physical examination, we suspected breast carcinoma in seven patients. The average size of the tumors was 39 mm (15-80 mm). Nine patients had a mammogram. In five cases, there was a suspicious breast mass mimicking a malignant tumor with an average size of 33 mm (25-60 mm). A ultrasonography was performed in 6 cases and revealed a suspicious ill-circumscribed nodule in four patients with an average size of 37.5 mm (10-60 mm). Five patients had a lumpectomy, and seven women underwent drainage of the abscess and the biopsy of its hull. The association of epithelioid cell granulomas and caseous necrosis was mandatory for the histological diagnosis of tuberculosis. All of them had an antitubercular therapy. The median period of follow-up was of 43 months (3-156 months). One patient presented with a recurrent abscess of the breast. CONCLUSION: Our study found that clinical examination and radiological imaging were not specific. Positive cultures for Koch bacillus or histological confirmation are mandatory for the diagnosis. A meta-analysis of the existing cases is needed.

[Radio-histological correlation of microcalcifications of the breast: a Tunisian center experience]. / Corrélation radio-histologique des microcalcifications du sein. Experience d'un centre Tunisien.

PURPOSE: Evaluation of surgical biopsy for mammary microcalcifications, in Tunisian patients according to the American College of Radiology's recommandations (ACR). MATERIAL AND METHODS: [corrected] 100 patients treated in Salah Azaiez Institute from January 2005 to December 2006, who underwent surgical biopsy for breast microcalcifications The diagnosis was based on mammography associated in 60% of the cases, to ultra-sonography. We correlated biopsies results to ACR classification. RESULTS: 123 surgical biopsies are related to ACR 3 lesions in 72 cases (58.5%) ACR 4 in 39 cases (32%) and ACR 5 in 14 case (11.5%). The histological examination showed malignant lesions in 4 cas/72 ACR3 (5.5%), 19/39 ACR4 (48.7%) and 10 among the ACR5 (71.4%). CONCLUSION: Our preliminary study introduces a diagnostic approach of mammary microcalcifications, that are usually infra-clinic lesions, in a general context of breast tumors detected with an average size of more than 3 cm.

[Typical medullary carcinoma of the breast: a retrospective study about 33 cases]. / Carcinome médullaire typique du sein : étude rétrospective à propos de 33 cas.

OBJECTIVE: Typical medullary carcinoma of the breast is a rare histological form of breast carcinoma. It represents less than 5% of all breast cancer. It is known for its favourable prognosis. Considering the rarity of the series we could retrieve, we aimed at underlining the particularities of this pathology. PATIENTS AND METHODS: Retrospective study about 33 cases of typical medullary carcinoma managed at the Salah-Azaïz Institute during a period of six years between 1994 and 1999. RESULTS: The mean age was 47.5 years. The left breast was concerned in about 55% of cases. Mean tumoral size was 46mm. Sixty-one percent of our patients were treated by radical surgery. Seventy-three percent undergo radiotherapy, 57% chemotherapy and 42% hormonotherapy. The five-year free-disease survival was about 85%. DISCUSSION AND CONCLUSION: Typical medullary carcinoma of the breast is a rare histological form of breast carcinoma. Its treatment is similar to the other breast cancers. Our results agree with the different published studies and confirm its favourable prognosis.

[Secondary breast cancer after treatment for Hodgkin's disease. About seven cases]. / Cancer du sein secondaire après traitement pour maladie de Hodgkin. A propos de sept cas.

OBJECTIVE: Therapeutic results of Hodgkin disease (HD) have improved by the use of combined radio-chemotherapy. However, this association can increase the risk of on-term effects including secondary cancers. In a retrospective study, we collected secondary breast cancer (BC) in patients previously treated with chemoradiotherapy for Hodgkin disease at Salah-Azaïz institute of Tunis. PATIENTS AND METHODS: Between 1975 and 2003, seven patients (six women and one man) treated for HD subsequently developed BC. Mean age at diagnosis of HD was 21 years (12-29). The first treatment was combined chemotherapy (MOPP-ABVD) and radiotherapy for all patients. Radiotherapy was delivered with cobalt 60 with large fields. The median dose was 41.3 Gy (2 Gy/fraction in 6 patients and 3.3 Gy in one). RESULTS: The breast tumours occurred after a median delay of 204 months (132-276). According to the TNM classification, we showed two stage T2, one stage T3, two stage T4b and two stage T4d. The mean clinical size was 47 mm (25-80 mm). All patients had infiltrating carcinoma. Axillary node histological involvement was found in 6 cases. All patients were treated by mastectomy and chemotherapy. Only one patient had a locoregional irradiation. Median survival was 26.5 months (12-48). Four patients died and three are still alive at respectively 24, 31 and 144 months. DISCUSSION AND CONCLUSION: According to the previous data, breast cancer represents 6.3 to 9% of all second cancers occurring after HD treatment. We conclude that especially young women and girls treated for HD should be carefully monitored. We suggest that secondary BC be sometimes treated by conservative radiosurgical approach.

[Benign schwannoma of the breast: about two cases]. / Schwannome mammaire bénin: à propos de deux cas.

Schwannoma is a rare nervous tumor developed on schwan cells. Only 22 cases of breast schwannoma have been published since 2005. It usually appears as a breast lump having clinical and radiological characteristics suggestive of kindness. Its diagnosis is histological. Its treatment is surgical. Through two observations and a review of the literature we would try to remind the characteristics of this tumor.

[Mammary tuberculosis: a retrospective study of 65 cases]. / Tuberculose mammaire: étude rétrospective de 65 cas.

OBJECTIVES: Remember the clinical, morphologic and histological particularities of mammary tuberculosis. Discuss clinical and radiological diagnostic problems caused by this localization, as far as breast cancer is concerned. PATIENTS AND METHODS: This is a retrospective study concerning 65 women suffering from mammary tuberculosis, diagnosed at Salah Azaiez Institute between January 1980 and December 2001. RESULTS: In this series, mammary tuberculosis represents 0.2% of the declared tubercular localizations and 0.3% of the mammary pathologies treated at the institute. The mean age of our patients was 36 years (19 to 79). Clinical findings were misleading. In 60% of cases the aspect was of a malignant tumor, in 24.6% of cases of a benign nodule and in 16.4% of cases of an abscess. The mammography concluded to a malignant lesion in 49% of cases. The fine needle aspiration achieved at 8 patients was negative in 6 cases and brought back the caseum in the 2 others. The diagnosis has been made, for all our patients, after histological study when we found typical tuberculosis lesions on pieces of tumorectomy, or biopsy. The diagnosis of tuberculosis was established; all patients had an anti-tuberculosis medical treatment in a department of infectious diseases out of the institute. DISCUSSION AND CONCLUSION: Clinical and radiological features of mammary tuberculosis are very confusing and cause a diagnostic problem with breast cancers. In front of symptoms evoking tuberculosis, biopsies must be done to eliminate an eventual cancer.

[Pelvic pseudotumoral actinomycosis with multisegmental lumbosacral root failure. A case report]. / Actinomycose pelvienne pseudotumorale avec atteinte multiradiculaire lombosacrée. A propos d'un cas.

We report a case of pseudotumoral pelvic actinomycosis with lumbosacral spinal involvement in a 21 year-old woman. The radiological work-up showed a pseudotumoral left ovarian lesion extended to sacral spine, which involved the fifth lumbar vertebra. Diagnosis of actinomycosis was established in histopathological examination of the tubo-ovarian mass under laparotomy. After four months of treatment with ofloxacin and rifampicin evolution was marked by improvement of general health and infection, associated to a progressive recovery of motricity of the lower limbs. Early diagnosis of pelvic actinomycosis may prevent the occurrence of rare but severe neurological complications of this disease.

[Relapse of gigantomastia after mammoplasty. Report of a case and literature review]. / Récidive d'une gigantomastie après mammoplastie. A propos d'un cas et revue de la littérature.

Gigantomastia is a very rare entity of undetermined aetiology that may be due to hormonal imbalance, decreased hormonal catabolism or hypersensitivity of the target organ. It poses the problem of surgical treatment, which can be exceptionally radical of necessity. We report a case of a 32-year-old woman of whom volume and vasculocutaneous complications required a simple bilateral mastectomy to treat a recurrence of gigantomastia occurring some months after a mammoplasty. Most of the cases of gigantomastia found in the literature are associated to the pregnancy or puberty and very rare cases of spontaneous gigantomastia were listed.

[Uterine adenomyosis. Clinical and therapeutic study. Report of 87 cases]. / Adénomyose utérine. Etude clinique et thérapeutique. A propos de 87 cas.

STUDY OBJECTIVE: The aim of our study is the analysis of Pound epidemiological, clinical, paraclinical and therapeutic aspects of adenomyosis. METHODS: A retrospective analysis was carried out on 87 patients who underwent hysterectomy for a variety of reasons (except for prolapse) between January 1, 1990, and December 31, 1997; and whose Histologic analysis of hysterectomy specimen revealed adenomyosis. RESULTS: 586 hysterectomy (except prolapse) was performed during the study period: frequency of adenomyosis in hysterectomy specimen is of 14.85%. The mean age of our patients is 43.97 years (Range: 26-64 years). 29.88% of them are postmenopausal and 41.37% had a past history of uterine trauma. Main symptoms were pelvic pain (71.22%) and abnormal uterine bleeding (82.77%). Preoperative diagnosis has been suspected on hysteroscopy in 63.22% of cases, hysterography in 58.46% and transvaginal sonography in 40.5%. A radical surgery first has been indicated in 57 cases, after failure of medical treatment in 16 cases and after failure of endometrial resection in 14 cases. Histologic analysis of the hysterectomy specimen shows associated myoma in 32.18% of cases, hyperplasia in 13.79%, polyps in 5.74% and atrophy in 3.44%. CONCLUSION: Adenomyosis, enigmatic disease, escapes always to a well-codified therapeutic strategy and bound closely related to hysterectomy. Earliest diagnosis could avoid the systematic evolution toward the radical treatments.