Retroperitoneal Solitary Fibrous Tumor: A "Patternless" Tumor.

INTRODUCTION: Solitary fibrous tumor is a rare type of mesenchymal, spindle-cell tumor reported mostly in the pleura. Retroperitoneal occurrence is rare and histopathological diagnosis is challenging. CASE PRESENTATION: A 55-year-old woman with nonspecific abdominal pain was found to have a retroperitoneal/pelvic mass adjacent to the upper rectum. The patient underwent surgical resection in clear margins of this pelvic tumor, entering the total mesenteric excision surgical plane. Final histopathology revealed a solitary fibrous tumor and the case is presented herein. DISCUSSION: Solitary fibrous tumor in the retroperitoneum is rarely found in the literature and to the best of our knowledge less than a hundred cases are described so far. Histopathological diagnosis is mostly based on a "patternless pattern" on microscopic examination, which is a storiform arrangement of spindle cells combined with a "hemangiopericytoma-like appearance" and increased vascularity of the lesion. Surgery is the mainstay of treatment and recurrence rates are generally low.

Skin metastases of vulvar squamous cell carcinoma. Presentation of a rare case.

Skin metastases secondary to vulvar carcinoma is an infrequent clinical entity. The authors describe a case of squamous vulvar carcinoma, which presented with cutaneous involvement as a part of distant spread. After a radical vulvectomy, bilateral inguino-femoral lymphadenectomy, and adjuvant radiotherapy, the patient developed multiple cutaneous metastases in lower extremities. This case was unique in presentation, with skin metastases secondary from vulvar carcinoma, and indicated advance disease and poor prognosis.

Collision tumor of the appendix: mucinous cystadenoma and carcinoid. A case report.

INTRODUCTION: Mucinous cystadenoma is the most common of benign neoplasms of the appendix and carcinoid is the most common type of primary malignant lesions of the appendix.We report a rare case of a 57-year-old female with combined mucinous cystadenoma and carcinoid tumor of the appendix.Dual carcinoid and epithelial neoplasia is a rare occurrence in the appendix. CASE REPORT: A 57-year-old Caucasian woman presented after incidentally palpating a mass on her right iliac fossa. Imaging modalities revealed a cystic tumor in the right iliac fossa with a diameter of about 8 cm, originating either from the right ovary or the appendix. She underwent laparoscopic surgical exploration, which revealed appendiceal mucocele and appendicectomy was finally performed as well as excision of a right ovarian cyst. Pathological examination showed acollision tumor consisting of mucinous cystadenoma and carcinoid tumor of the appendix. Because of the size and extension of the carcinoid tumor, which the pathology report revealed, she underwent re-exploration and laparoscopic right colectomy. DISCUSSION: Mucinous cystadenoma is rare, but it is the commonest of benign appendiceal tumours accounting for 0.6% of appendectomy specimens. It can present as appendicitis,mucocele or if the tumour ruptures, as pseudomyxomaperitonei. On the other hand, carcinoid is the most common type of primary malignant lesion of the appendix and 0.3-0.9%of appendectomy specimens, with small predominance in female patients. Rare cases of mucinous cystadenomas of the appendix coexisting with carcinoid tumors were reported before, but in our case it was a collision tumor with no transitional zone between them. Also, the clinical presentation of our patient and the differential diagnosis of ovarian lesions from appendiceal tumors is worth being mentioned.

Granulosa cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 21 cases.

PURPOSE: To further study the clinicopathologic and immunohistochemical features of ovarian granulosa cell tumors (GCTs). METHODS: We retrospectively studied all cases of GCTs diagnosed in our laboratory over the last 10-year period. Immunohistochemistry for inhibin, vimentin, cytokeratin, Ki-67 and p53 was performed on archival paraffin blocks. Pathologic and immunohistochemical findings were correlated with the clinical records of the patients. RESULTS: Twenty-one cases (15 of the adult and 6 of the juvenile type) were retrieved. All patients were FIGO Stage I at the time of diagnosis. Recurrent disease was detected in four patients (19%) during a median follow-up of 36 months (range 2-26 years). Pathology revealed a concomitant theca-cell component in three cases, a Sertoli-Leydig component in one case, and a thecoma in one case. Archival tissue material was available in 12 cases. Immunohistochemistry was positive for: beta-inhibin in 12/12 cases (100%), vimentin in 11/12 cases (91.7%), cytokeratin in 3/12 cases (25%), CD34 in 0 cases (0%), and p53 in 2/12 cases (16.7%). The Ki-67 index was < 5% in 12/12 cases (100%). No significant correlations were observed between the pathologic and immunohistochemical parameters examined and the clinical outcome. CONCLUSIONS: Despite the relatively indolent nature and favorable prognosis of most GCTs, late recurrences are not a rare event even in Stage I patients, necessitating a close and long-term follow-up. The identification of novel prognostic markers, in addition to our traditional staging parameters such as clinical staging, is needed in order to more accurately predict probabilities of recurrence in these patients.

Parathyroid cysts of the neck and mediastinum. Case report.

Five cases of parathyroid cyst are presented and the literature concerning this rare entity is reviewed. Three of the five cysts were located in the neck and were non-functioning. The other two were in the anterior mediastinum and functioning, and indeed were associated with hypercalcemic crisis. Four cases were treated surgically, and in one non-functioning parathyroid cyst both diagnosis and treatment were accomplished with fine-needle aspiration. The cystic appearance, color of the fluid contents, high content of parathyroid hormone and histologic features distinguish parathyroid cyst from the much commoner thyroid cyst. Non-functioning parathyroid cysts can be treated with fine-needle aspiration, whereas functioning cysts require surgical removal.

Extraskeletal Ewing's sarcoma in a pregnant woman: a case report.

Extraskeletal Ewing's sarcoma is an unusual form of soft-tissue sarcoma. We report a case of a pregnant woman with a rapidly growing tumour of the left thigh, who went into complete remission with combination chemotherapy. Aggressive therapy is justified in this kind of tumour even in cases with advanced disease.

Computed tomography in the evaluation of spinal aneurysmal bone cyst.

Computed tomographic findings in three cases of aneurysmal bone cyst (ABC) are reported. Although conventional radiography is usually adequate in the demonstration of an expansile osteolytic cavity, CT may provide additional information about tumour characteristics, localization and extent, show the lesion's relation to the spinal cord and thus be of great help in preoperative planning.

Congenital fibrosarcoma metastatic to the choroid.

A 2 1/2-year-old boy developed a choroidal metastasis from a congenital fibrosarcoma of the lower left limb that had been amputated shortly after birth. To our knowledge this is the first reported case of a congenital fibrosarcoma that metastasized to the choroid.

Vasculature of the optic nerve in anencephaly.

The optic nerves and globes obtained from 6 anencephalics were studied histologically and compared to normal specimens obtained from 4 stillborn infants as well as 1 case of septo-optic dysplasia. Special emphasis was placed on examination of the optic nerve, and it was found that an average of 48 vessels per high-power field were seen posterior to the lamina cribrosa in anencephalics. In contrast, control globes had an average of 12 vessels per high-power field. This suggested that an increase in the vasculature of the hypoplastic optic nerve is a characteristic feature of anencephalics.

The visual results after vitreous loss during cataract extraction.

We followed the progress of 50 eyes in which cataract extraction was complicated by vitreous loss for 6 to 48 months and matched them with 50 eyes that had not lost vitreous. The results show that the number of eyes attaining 6/6 (20/20) or 6/7.5 (20/25) vision is significantly higher in the controls. All eyes that had vitreous loss retained useful vision. A comparison between our cases managed by vitrectomy and those of vitreous loss without vitrectomy reported in the literature, showed that vitrectomy gave better visual results with fewer complications.

Angiomyxoma of umbilical cord.

A rare angiomyxoma of umbilica cord occurred in a male infant. The presence of such a tumor with its vascular component arising from a hypoplastic umbilical artery supports the hypothesis that the lesions arose through improper placental vascularization.

The visual field in indirect traumatic rupture of the choroid.

Nine cases of indirect rupture of the choroid due to trauma were examined by indirect ophthalmoscopy, fluorescein angiography and static and kinetic perimetry. Our results indicate that the visual field defect cannot be correlated with the ophthalmoscopic picture as concerns shape, size, or location of the lesion. Seven of the 9 cases had a central scotoma and we demonstrated nerve involvement in 4 of the 9 cases. We do not know of other studies of the visual field in indirect traumatic rupture of the choroid.