Extragastrointestinal stromal tumour of the lesser omentum: A case report and literature review.

INTRODUCTION: Extragastrointestinal stromal tumours (EGISTs) are very uncommon compared to their gastrointestinal counterparts. Most of them originate from the intestinal mesentery and the omentum. CASE REPORT: A 70 year-old Caucasian woman presented with a bulky abdominal mass which on laparotomy was found to originate from the lesser omentum and was completely resected. Histological examination revealed spindle cells with severe pleomorphism and high mitotic activity. Immunohistochemically, the tumour cells showed strong positivity for c-kit (CD117), DOG-1 and human haematopoietic progenitor cell antigen (CD34). An exon 11 deleterious mutation was identified and thus regular dosing of 400mg imatinib mesylate was initiated. DISCUSSION: There have been only a few previous reports of EGISTs arising in the lesser omentum. Although EGISTs seem to have morphological and immunohistochemical similarities with GISTs, their pathogenesis, incidence, genetic background and prognosis are not completely known because they are extremely rare. It is strongly believed that such tumours originate from cells, which have similar pathological characteristics and biological behaviour as the intestinal cells of Cajal. In most series of EGISTs, a female predominance, a greater size and a higher mitotic index than GISTs were observed. CONCLUSION: EGISTs are very rare mesenchymal tumours which originate from cells outside the gastrointestinal tract and tend to have a more aggressive biological behaviour than their GI counterparts. Complete surgical resection is the most effective treatment associated with the use of imatinib in the presence of adverse prognostic factors. In any case a strict follow-up is necessary due to high recurrence rates.

Uterine angioleiomyoma causing severe abnormal uterine bleeding.

BACKGROUND: Angioleiomyoma or angiomyoma or vascular leiomyoma is an unusual benign mesenchymal neoplasm. The authors present a rare case of large uterine angioleiomyoma causing severe abnormal uterine bleeding. CASE: The patient, a 53-year-old, gravida 2, para 2, premenopausal Greek woman presented with a complaint of severe abnormal uterine bleeding. On gynecologic examination there was a palpable pelvic mass. Preoperative computer tomography (CT) of the abdomen and pelvis revealed an intra-abdominal mass 25 x 15 cm with abnormally increased vascularization. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, total omentectomy and elective pelvic lymph node dissection. Histopathology revealed uterine angioleiomyoma. Follow up 84 months after initial surgery showed no evidence of recurrence. CONCLUSION: Despite the type of surgery, patients with uterine angioleiomyoma have very low risk of recurrence and excellent prognosis.

Uterine malignant mixed Müllerian tumor after adjuvant tamoxifen treatment for breast cancer.

BACKGROUND: Uterine malignant mixed Müllerian tumor (MMMT), also known as carcinosarcoma, is a biphasic tumor of the female genital tract and demonstrates both malignant epithelial (carcinoma) and mesenchymal (sarcoma) components. The authors present two cases of uterine MMMT after adjuvant tamoxifen (TAM) treatment for breast cancer and a review of the current literature. CASES: The patients presented with a complaint of abnormal uterine bleeding. They both had a history of breast cancer Stage IIB previously treated with modified radical mastectomy, at 51 and 78 months, respectively. They also had history of tamoxifen treatment 20 mg daily for seven and 73 months respectively. They underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and total omentectomy. Histopathology revealed a uterine MMMT. Postoperatively, they received adjuvant chemotherapy and radiotherapy. One of the patients died 26 months after initial surgery due to uterine MMMT. CONCLUSION: Uterine MMMT is a rare, highly-aggressive, and rapidly-progressing tumor associated with a poor prognosis. Postmenopausal patients, with prolonged adjuvant TAM treatment for breast cancer, are at increased risk for the development of uterine MMMT.

Primary ovarian leiomyosarcoma.

BACKGROUND: Primary ovarian leiomyosarcoma is an extremely rare subtype of ovarian sarcomas. It most commonly occurs in postmenopausal women and has unfavorable prognosis. CASE: The patient, a 58-year-old postmenopausal woman, presented with a complaint of abdominal pain. Preoperative examination revealed an intraabdominal mass 25 x 17 x 14 cm in the right adnexa. She underwent bilateral salpingo-oophorectomy, total omentectomy, appendectomy and bilateral pelvic lymphadenectomy. The histopathology revealed leiomyosarcoma of the right ovary Stage Ia. She did not receive any postoperative adjuvant therapy. Follow-up 21 months after initial surgery, showed no evidence of recurrence. CONCLUSION: Additional studies are needed to understand more about the nature, clinical behavior and treatment of this very rare tumor.

Synchronous squamous cell carcinoma of the endometrium and endometrioid adenocarcinoma of the ovary.

BACKGROUND: Synchronous primary endometrial and ovarian cancers are relatively uncommon in general population. The etiology and pathogenesis of this phenomenon remains unclear. The authors' aim was to present a case of synchronous squamous cell carcinoma of the endometrium and endometrioid adenocarcinoma of the ovary and review current literature. CASE: The patient, a 64-year-old, nulliparous postmenopausal Greek woman presented with a complaint of abdominal pain and abnormal uterine bleeding. Preoperative computer tomography (CT) of the abdomen and pelvis, and abdominal ultrasound (U/S) revealed an intra-abdominal three cm mass with solid components between the left ovary and small bowel. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAH+BS), total omentectomy, pelvic and para-aortic lymph node dissection, and removal of the implant at the serosa of small bowel. Histopathology revealed Stage IA endometrial cancer squamous type and Stage IIIC ovarian cancer of endometrioid-type. Postoperatively the patient underwent adjuvant chemotherapy and radiotherapy. Follow-up of 22 months after initial surgery revealed no evidence of recurrence. CONCLUSION: The reason for better median overall survival of patients with synchronous primary endometrial and ovarian cancers is not intuitively obvious. Perhaps favourable clinical outcome may be related with the detection of patients at early stage and low-grade disease with an indolent growth rate.

Loop electrosurgical excision procedure in Greek patients with vaginal intraepithelial neoplasia and history of cervical cancer.

OBJECTIVE: The aim of our study was to evaluate the therapeutic effectiveness of loop electrosurgical excision procedure (LEEP) in Greek patients with vaginal intraepithelial neoplasia (VAIN) and history of cervical cancer. MATERIALS AND METHODS: Between January 2002 and January 2009, eight women with histologically confirmed VAIN and history of cervical cancer were included in our study. For the LEEP procedure we used a high frequency Electrosurgery Unit with at least 80 W output. RESULTS: Complete response rate, at 12 months of follow-up, was 75%. Recurrence rate, at 12 months of follow-up, was 25%. Complete response rate, at 24 months of follow up, was 62.5%. Recurrence rate, at 24 months of follow up, was 37.5%. CONCLUSION: LEEP may constitute a valuable excisional method for the treatment of VAIN in cases with a history of cervical cancer. It provides an interpretable specimen of the whole lesion within a few minutes. It needs a short period of training and has low cost.

Proliferative Brenner tumor of the ovary. clinicopathological study of two cases and review of the literature.

BACKGROUND: Ovarian Brenner tumors are rare epithelial tumors that account for 1%-2% of all ovarian neoplasms. They can be subdivided into benign, borderline or proliferative, and malignant neoplasms. In the vast majority of cases, these lesions are benign. Tumors of borderline malignancy are less frequent and only about 1% of Brenner tumors are malignant. We present two cases of Brenner tumors with borderline malignancy which were treated in our Department together with a review of the literature. CASES: A 50-year-old, gravida 1, para 1, patient was admitted for abnormal vaginal bleeding. Clinical examination, abdominal ultrasound (US), and computed tomography (CT) revealed a cystic multilobulated tumor of the right ovary with solid elements measuring 20 x 19 x 15 cm in diameter. In the other case a 70-year-old, gravida 2, para 2, patient presented with severe urinary difficulties. Palpation revealed a mobile abdominopelvic tumor 10 x 15 in diameter. US and CT exhibited a cystic tumor with multiple solid elements and calcifications of the left ovary. Both patients underwent exploratory laparotomy. Total abdominal hysterectomy with bilateral salpingo-oophorectomy and total omentectomy were performed in both cases, while pelvic lymphadenectomy was decided only in the second case. Histologically, in both cases the diagnosis confirmed borderline Brenner tumor. CONCLUSION: Although Brenner tumors are rare and the majority of them are benign, the correct histological diagnosis at frozen section with identification of the small proportion of malignant tumors, allows the extent of the operation to be adapted if needed.

Angiokeratoma of the vulva.

Angiokeratoma of the vulva is relatively uncommon in the general population. We present two cases of angiokeratoma of the vulva and review the literature. The two patients presented with the complaint of vulvar pruritus. They underwent wide local excision of the lesions. Histopathology revealed angiokeratoma of the vulva. The women remain well with no evidence of recurrence 48 and 32 months after initial surgery. Although it is a rare disease, angiokeratoma of the vulva should be included in the differential diagnosis of a vulvar tumor.

Primary ovarian non-Hodgkin's lymphoma.

BACKGROUND: Primary ovarian non Hodgkin's lymphoma (PONHL) is a very rare disease. We present a case of PONHL and review the literature. CASE: The patient, a 24-year-old nulliparous Greek woman, presented with the complaint of abdominal pain. She underwent left salpingo-oophorectomy, multiple biopsies from the right ovary, total omentectomy, pelvic and paraortic lymphadenectomy, appendectomy and curettage. The histopathology revealed diffuse large B-cell non-Hodgkin's lymphoma of the left ovary. She underwent postoperative chemotherapy. She remains well without evidence of disease, 15 months after initial surgery. CONCLUSION: The use of chemotherapy is based on the principle that PONHL must be considered a localized manifestation of systemic disease. Patients with PONHL have a similar outcome compared to patients with other NHL.

Loop electrosurgical excision procedure in Greek patients with vaginal intraepithelial neoplasia.

OBJECTIVE: The aim of our study was to evaluate the therapeutic effectiveness of the loop electrosurgical excision procedure (LEEP) in Greek patients with vaginal intraepithelial neoplasia (VAIN). MATERIALS AND METHODS: Between January 2002 and January 2009, 23 women with histologically confirmed VAIN were included in our study. For the LEEP procedure we used a high frequency electrosurgery unit with at least 80 W output. RESULTS: Complete response rate at 12 months of follow-up was 86.96%. Recurrence rate at 12 months of follow-up was 13.04%. Complete response rate at 24 months of follow-up was 75%. Recurrence rate at 24 months of follow-up was 25%. CONCLUSION: LEEP may constitute a valuable excisional method for the treatment of VAIN. It provides an interpretable specimen of the whole lesion within a few minutes. It needs a short period of training and has low cost.

Synchronous primary endometrial and fallopian tube cancers.

BACKGROUND: Synchronous primary cancers are relatively uncommon in the general population. We present a case of synchronous primary endometrial and fallopian tube cancers and review the literature. CASE: The patient, a 54-year-old, gravida 2, para 2 postmenopausal Greek woman presented with a complaint of abnormal vaginal bleeding. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and total omentectomy. The histopathology revealed synchronous primary cancers of the endometrium and right fallopian tube. The patient underwent postoperative chemotherapy and postoperative radiotherapy. She remains well without evidence of disease, 65 months after initial surgery. CONCLUSION: The reason for the better median overall survival of patients with synchronous primary endometrial and fallopian tube cancers is not intuitively obvious. Perhaps it is due to the detection of patients at earlier clinical stage and lower grade disease state.

Overexpression of matrix-metalloproteinase-9 in human breast cancer: a potential favourable indicator in node-negative patients.

Matrix metalloprotease-9 (MMP-9; 92 kDa type IV collaganase, gelatinase B) is regarded as, important for degradation of the basement membrane and extracellular matrix during cancer invasion and other tissue-remodelling events. In this study we evaluate the prognostic value of MMP-9, by immunoperoxidase staining in a series of 210 breast cancer tissues. The results were quantitated using the HSCORE system, which consider both staining intensity and the percentage of cells stained at given intensities. MMP-9 status was compared with the concentration of cytosolic Cathepsin-D and with other established prognostic factors, in terms of disease free survival and overall survival. The median follow-up period was 62 months. MMP-9 staining was observed primarily in cancer cells, and to a lesser degree in surrounding stromal cells. MMP-9 expression was not detected in normal breast tissue. Levels of MMP-9 expression below the cut-off point were more frequently observed in larger (P = 0.014), invasive ductal histologic (P = 0.037), progesterone receptor (PR)-negative and PR-strong positive tumours (P< 0.001), as well as samples belonging to patients with stage III-IV disease (P = 0.009) and age 45-55 years (P = 0.011). In univariate analysis, node-negative breast cancer patients with tumors positive for MMP-9 had a considerable reduction in risk for relapse (RR = 0.45;P = 0.039) or death (RR = 0.32;P = 0.009). Multivariate analysis indicated that MMP-9 status was an independent favourable predictor of OS (RR = 0.47;P = 0.034) in node-negative but not in node-positive patients. Our results suggest that MMP-9 may be an independent favourable prognostic factor in node-negative breast cancer patients. The overexpression of MMP-9 in breast cancer may be also used as a marker to subdivide node negative breast cancer patients in order to determine the optimal treatment modality.

Congenital cystic adenomatoid malformation in an adult presenting as lung abscess.

The case of a 21-year-old male with congenital cystic adenomatoid malformation is presented. His medical history started after his birth with recurrent pulmonary infections during his infancy. Lung abscess of the right lower lobe was suspected and right lower lobectomy was performed to remove a sizeable mass infiltrating the largest part of the lobe. The clinical features, diagnostic procedures, differential diagnosis, pathologic characteristics, therapeutic assessment, etiopathology and prognosis of the tumor are discussed.

Inflammatory pseudotumor: a controversial entity.

Inflammatory pseudotumor of the lung is considered to be a rare, benign, neoplastic lesion, consisting mainly of spindle mesenchymal cells, sometimes in such a way that its histological appearance mimics that of a spindle cell sarcoma, fibrous histiocytoma or fibrosarcoma. On the occasion of a case managed in our department, the literature is reviewed, in an attempt to clarify some issues concerning this tumor. Emphasis is given to complete resection of the tumor for both diagnostic and therapeutic purposes. Malignant behavior may occur and recurrence is possible.