1/25. Metastatic leydig cell tumor with sarcomatoid differentiation. Leydig cell tumors of the testis are uncommon. Only about 10% of cases have a malignant course. It has been stated that the only definite criterion for malignancy is presence of metastasis. We present a 47-year-old patient with metastatic leydig cell tumor 17 years after initial diagnosis, to our knowledge the longest reported interval between diagnosis and the development of metastasis. The primary tumor did not exhibit convincing features of malignancy. The initial metastasis in the right perirenal fat tissue showed a biphasic tumor with sarcomatoid differentiation not described previously in a metastatic Leydig cell tumor. ( info) |
2/25. Uterine adenolipoleiomyoma: a rare hamartomatous lesion. An apparently unique intramural uterine lesion is described for which we propose the name adenolipoleiomyoma. On gross examination, a well-circumscribed white intramural mass contained focal fatty areas. histology showed the mass to be composed of smooth muscle, adipose tissue, and endometrial, endocervical, and tubal type glands. The endometrial glands were surrounded by typical endometrial stroma. There was no evidence of adenomyosis in the uterus outside the lesion. This is only the second report of such a lesion within the uterus and the first with an intramural location. We believe it to be a benign hamartomatous lesion. ( info) |
| We report two cases of lipoblastoma with chromosome 8-related aberrations, ie, a 92,XXYY,t(7;8Xp22;q11.2)x2 [8]/46,XY[16] in Case 1 and a 46,XY,-8,-13,add(16) (q22), mar, r [cp13]/46,XY[7] in Case 2. Using spectral karyotyping and fluorescence in situ hybridization techniques, the karyotype of Case 2 was redesignated as 46,XY, r(8), del(13)(q12), der(16)ins(16;8)(q22; q24q11.2)[cp13]/46,XY[7]. This report delineates a new chromosome rearrangement, ie, der(16)ins(16;8)(q22; q24q11.2) in lipoblastoma, and also confirms the t(7; 8)(p22;q11.2), reported only once previously, as a recurrent translocation involved in such a tumor. These findings provide valuable information for clinical molecular cytogenetic diagnosis of lipoblastoma. Furthermore, this report highlights the value of cytogenetic and molecular cytogenetic analysis in differential diagnosis of childhood adipose tissue tumors and adds to the number of lipoblastomas reported with chromosomal abnormalities at 8q11.2. ( info) |
4/25. Adenomyolipoma of the uterus: a case report. Adenomyolipoma of the uterus is a rare, benign, polypoid lesion considered to be of hamartomatous origin or represent an unusual type of benign Mullerian mixed tumour with a heterologous element. The authors present a case of uterine adenomyolipoma and discuss its pathogenesis. A 62-year-old woman complained of lower abdominal pain and postmenopausal bleeding. Imaging techniques revealed a solid ovarian mass and a polypoid intrauterine lesion. The frozen section diagnosis of the ovarian mass was a thecoma. A total hysterectomy and bilateral salpingo-oophorectomy were performed. On gross examination a pedunculated, polypoid lesion of 7x4.5x3cm was found in the uterine cavity. Microscopically, the polypoid lesion contained both epithelial and mesenchymal elements. The epithelial elements were endometrial glands of various size, formed by proliferative endometrial cells. The mesenchymal elements were composed of endometrial stroma, smooth muscle and mature adipocytes. Both the epithelial and the mesenchymal elements showed a benign appearance, were intermingled with each other and periglandular stromal condensation was absent. The lesion had an irregular surface. Microscopic diagnosis was an adenomyolipoma. The peculiar shape and microscopic features of this lesion suggested that it was a variant of benign Mullerian mixed tumour. ( info) |
5/25. Laparoscopic excision of a solitary renal cell carcinoma metastasis to the contralateral perirenal adipose tissue. Metastasis of renal cell carcinoma to the contralateral perirenal fat is a very rare occurrence. We report a case of a synchronous, solitary perirenal metastasis excised laparoscopically 2 years after initial, open radical nephrectomy. ( info) |
6/25. Lipomatous variant of nasopharyngeal angiofibroma: a case report. Nasopharyngeal angiofibroma is an uncommon benign vascular tumor that occurs in adolescent boys and young men. We describe an unusual histological variant of this entity characterized by a prominent adipose tissue component. The patient was a 35-year-old man with a 20-year history of nasal obstruction. Results of physical examination and angiography demonstrated a nasal mass consistent with a nasopharyngeal angiofibroma. We discuss the differential diagnoses pathologically for this lesion. ( info) |
7/25. Laparoscopic resection of a large periadrenal nonmalignant pheochromocytoma. This is, to our knowledge, the first case description of an extraadrenal pheochromocytoma located in the periadrenal fat. pheochromocytoma is a tumor that originates in the chromaffin tissue. Extraadrenal pheochromocytomas have been described commonly in locations such as the organ of Zuckerkandle (29%); the bladder (12%); the sacrum, testis, rectum, and pelvic floor (2%); the upper abdomen in association with celiac, superior mesenteric, and inferior mesenteric ganglia (43%); the thorax (12%); and the neck (2%), most commonly in association with the ninth or tenth cranial nerve ganglion. Our patient was a 40-year-old woman known to have had an adrenal mass for the last 4 years. She was referred for surgery because of an increase in the size of the mass to 11 cm. Laparoscopic adrenalectomy was performed via a posterior flank approach. The pathology report was of periadrenal fat pheochromocytoma, with positive staining for synaptophysin, chromogranin, and vimentin. The patient was discharged on postoperative day 3. The unique feature in this case was the uncommon location of the extraadrenal tumor: the supraadrenal fat. The other unique finding in this case was that the pheochromocytoma was neither symptomatic nor malignant, common features of extraadrenal masses. ( info) |
8/25. Lipoblastomatosis of the neck causing hemiparesis: a case report and review of the literature. BACKGROUND: lipoblastoma and lipoblastomatosis are rare pediatric adipose tumors that sometimes affect the neck or spinal cord. This case is the third report of lipoblastoma extending into the spinal canal, the first report of intradural tumor extension, and the first report of hemiparesis resulting from lipoblastoma compressing the spinal cord. methods: A 13-month-old boy was seen by a pediatrician for a firm, supraclavicular neck mass on the left side. After being evaluated by CT and MRI scanning, the tumor was partially resected. RESULTS: Postoperative microscopic examination of the tumor showed adipose cells with mature nuclei and well-formed fat vacuoles interspersed with fibrovascular septa, a finding consistent with maturing lipoblastoma. CONCLUSIONS: Although lipoblastomatous tumors are treated with complete surgical resection when possible, location of these tumors in the neck may dictate partial resection to avoid intraoperative injury to the spinal cord. ( info) |
9/25. lipoblastoma in a 23-year-old male: distinction from atypical lipomatous tumor using cytogenetic and fluorescence in-situ hybridization analysis. lipoblastoma is a rare benign tumor that occurs primarily in infancy and early childhood. We present the rare presentation of a 12 cm sized intramuscular lipoblastoma of the thigh in a 23-year-old patient. On histology, the tumor strongly resembled an atypical lipomatous tumor due to the presence of lipoblasts and atypical stromal nuclei. The very focal lobulation and myxoid change presented the only histological hint towards a lipoblastoma. Cytogenetic and subsequent FISH evaluation of the tumor cells showed a 46,XY, t(8;15) (q12;q25) as chromosomal change with rearrangement of the PLAG1 gene. The present case indicates that lipoblastoma should enter the differential diagnosis of an 'atypical' deep seated fatty tumor in adults. The diagnostic value of cytogenetic/molecular analysis in the differential diagnosis of lipomatous tumors is underscored as well. ( info) |
10/25. ring chromosomes and low-grade gene amplification in an atypical lipomatous tumor with minimal nuclear atypia. Atypical lipomatous tumors (ALTs) are characterized by supernumerary ring chromosomes and/or giant marker chromosomes, which typically are composed of interspersed, amplified 12q-sequences, are C-band negative, lack alpha-satellite sequences, and display high copy numbers of several oncogenes, including HMGA2 (a.k.a. HMGIC) and MDM2, from the 12q13-15 region. In the present study, we report the cytogenetic and molecular genetic findings in an ALT with minimal nuclear atypia from a 16-year-old boy. At G-banding analysis, 1-3 supernumerary ring chromosomes were detected. Combined binary ratio labeling fluorescence in situ hybridization (COBRA-FISH) showed that the rings were entirely composed of material from chromosome 12, and by further FISH analysis with locus-specific probes it was revealed that they consisted of two tandemly arranged copies of the segment 12p11.2-p13.2 to 12q21.2-q23.1. Within that segment of chromosome 12, there was a small deletion including the HMGA2 locus. There was no variation in ring size and no interphase bridges could be detected, indicating that the ring chromosomes were mitotically relatively stable. The present case thus adds support to the concept that there exists a subset of ALT with limited or minimal nuclear atypia and low-level amplification of 12q sequences, further suggesting the possibility of a molecular genetic continuum between lipoma and classical examples of ALT. Furthermore, the present data strongly imply that it is the composition of the rings rather than the ring chromosome formation as such that causes the genetic instability and nuclear atypia frequently seen in ALTs. ( info) |