We report a case of dedifferentiated liposarcoma of retroperitoneum as a recurrent form in a 41 year old male. The patient received a extirpation for retroperitoneal mass and diagnosed as myxoid liosarcoma 4 years ago. The patient experienced 3 recurrences over a period of 4 years and diagnosed as myxoid liposarcoma in the second, third recurrence also. Histologically, the mass was composed of several clearly distinct elements : well differentiated liposarcoma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma, poorly differntiated sarcoma, and fibrosarcoma. Immunohistochemically, S-100 protein was expressed in the area of spindle cell sarcoma, well differentiated liposarcoma, and malignant fibrous histiocytoma but alpha-1-antichymotrypsin was only expressed in the area of myxoid malignant fibrous histiocytoma.

Spindle cell carcinoma of the upper aerodigestive tract mucosa was usually presented as polypoid mass and shows squamous cell carcinoma or dysplasia in the surface and underlying spindle cell proliferation. The spindle cell area discloses a variable pattern of sarcoma including rare osteosarcoma of 0~20% incidence. The histogenetic origin of the spindle cell component is now considered a metaplasia of squamous cell carcinoma. We experienced a case of spindle cell carcinoma of larynx showing massive underlying bone formation with proliferation of osteoclast-like cells in 75 year-old man. The immunohistochemical study demonstrates positive reaction with cytokeratin in area of squamous cells and with vimentin in area of spindle cells and osteoclasts. There are very focal reactivity for high molecular weight cytokeratin in spindle cell area.
Incidence

Microcystic adenxal carcinoma is an unusual, locally aggressive neoplasm that has recently been recongized as a clincopathologic entity. Its histologic appearance includes both pilar and eccrine differentiation. Microscopically, the tumor consisted of small cysts and gland-like structures in superficial portion. In other area, basaloid cell nests and abortive hair follicles in the sclerotic stroma were seen. The cysts were filled with secretory eosinophilic material, which was positively stained with Periodic acid-Schiff and carcinoembryonic antigen. Immuno-peroxidase staining for carcinoembryonic antigen supported the dual differnetiation of this neoplasm. Despite the benign histologic appearance, there was deep and extensive infiltration of the subcutaneous tissue.
Cysts

The Pleomorphic xanthoastrocytoma(PXA) is considered as a special subgroup of gliomas because of its distinctive characteristics: onset in young subject; predilection for the temopral or parietal lobe and a superficial location; frequent appearance as a yellow encapsulated mass with a grossly visible tumor-associated cyst; marked histological pleomorphism; little or no mitosis and no necrosis; presence of a rich reticulin network; and demonstrable GFAP in many of the fusiform and giant cells; most importantly, the relatively favorable prognosis despite plemorphism and bizzare giant cells in the microscopic picture. The objective of this report is to add one more case of pleomorphic xanthoastrocytoma to the medical literature.

Hamartoma of the bladder is quite a rare entity which is composed of a disorderly admixture of mature cellular elements normally present in the urinary bladder. There is a great controversy regarding the pathogenesis of this lesion. Whether it is a true hamartomatous lesion or metaplastic lesion developed secondary to the inflammatory process. Similar or identical lesions has often been given by other names such as florid examples of cystitis glandularis. We prefer to cell florid examples of cystitis glandularis rather than hamartoma when it was occurred in an old age higher then 50th decade. Here we report a case of hamartoma of the urinary bladder in 44 years old man. Cystoscopic examination revealed a papillary polypoid mass which was attached to the fundus of bladder by long stalk. The mass measured 1.5 cm in greatest diameter. It was composed of epithelial nests resembling von Brunn's nest, cystitis glandularis or cystitis cystica dispersed in a stroma rich in smooth muscle and fibrous tissue.
Hamartoma

Compared with osteosarcoma of bone, primary osteosarcoma of the soft tissue is very rare. Extraskeletal osteosarcoma is also a highly malignant pleomorphic sarcoma composed of cells exhibiting primarily osteoblastic, and to a lesser extent, chondroblastic differentiation. A case of extraskeletal osteosarcoma in the right sole is presented in a 67 year old male. The patient had noticed a progressively enlarging soft tissue mass, during about 14 months. This sarcoma was located in the soft tissues without attachment to the skeleton, as determined by examination of the X-Ray findings. The mass of right plantar portion was simply excised and pathologically confirmed to be an extraskeletal osteosarcoma. The clinical and pathological features of this sarcoma are described, and brief review of the literature is made.

In contrast to the nodular renal blastema which is defined by nests of primitive metanephric cells after 36 weeks of gestation, the nephroblastomatosis is characterized by neoplastic proliferation of the primitive cells. This lesion is presumed to be closely related to the development of Wilms' tumor. We report a case of bilateral nephroblastomatosis associated with Wilms' tumor in a child. This 4 1/2 year-old girl was admitted because of a 10 cm-sized round mass in the right kidney, and smaller nodules in the left kidney and the lung. After three cycles of chemotherapy and subsequent disappearance of the nodules in the left kidney and lung, she underwent a right nephrectomy and a wedge resection of the left kidney. A round Wilms' tumor mass was seen in the lower pole of the right kidney. Remaining right renal cortex showed multiple, slightly depressed gray-white nodules associated with multiple samll cysts. They were comprised of multifocal subcapsular nests of primitive nephrogenic cells with focal tubular or glomerular differentiation. They resembled fetal renal tissue. In the left kidney, similar nests of primitive cells were also noted. These lesions were interpreted as multifocal perilobar type of nephroblastomatosis.
Child ; Male ; Female ; Humans

We report a case of angiosarcoma of the jejunum in a 65-year-old female. This angiosarcoma developed 20 years after postoperative irradiation for cervical carcinoma of the uterus. Grossly, the resected jejunum showed a 8 cm long segmental hemorrhagic lesion with multiple, small hemorrhagic nodules or cysts on its serosa and mesentery. Microscopically, the wall of jejunum was infiltrated by epithelioid or polygonal tumor cells arranged in solid nests or lining irregular vascular spaces. The case is of interest that the tumor occurred in the very unusal site for angiosarcoma and the patient had a past history of irradiation for uterine cervical carcinoma 20 years ago. In addition, the resected intestine showed histologic changes of chronic irradiation effect. Therefore, this case supports the view that there is cause and effect relationship between irradiation adn angiosarcoma.
Female ; Humans ; Cysts

Gastric lesion in familial polyposis coli is commonly presented with fundic gland hyperplastic polyps, but duodenal and gastric adenomas together with their carcinomatous transformation have been rarely described in familial polyposis coli mostly by case reports. We present three cases of gastric adenomas in familial polyposis coli with one in synchronous development of gastric adenocarcinoma in Korea. All three cases had the family history related to familial polyosis coli and received proctocolectomy because of synchronous development of colonic adenocarcinoma. One patient developed gastric polyposis and adenocarcinoma 8 years after colectomy, and the remaining two presented with multiple polyps either in the stomach or duodenum synchronously at the time of the diagnosis of familial polyposis coli with colonic adenocarcinoma. None disclosed any evidence of Gardner's syndrome. We conclude that association of gastric adenomas in familial oplyposis coli is not uncommon and gastric adenoma-carcinoma sequence is an another important participating mechanism to understand the histogenesis of gastric carcinoma in Korea.
Adenocarcinoma ; Adenoma

Dedifferentiated chordoma is a rare pathologic entity presenting an additional sarcomatous component in otherwise classical chordoma. It has been also emphasized that this neoplasm is classified as a distinct entity because of its different clinical settings and aggressive behavior. Dedifferentiation is a peculiar phenomenon but its histogenesis has remained controversial. A 50-yera-old man developed a huge tumor mass in the retrorectal, presacral area, featured with two histological components. The one was a typical chordoma accounted for approximately 60% of the mass and the other was made up of highly cellular, plemorphic, undifferentiated tumor cells, reminiscent partly to the cells of plemorphic malignant fibrous histiocytoma. Ultrastructural features and immunoreactivity against cytokeratin, S-100 protein and alpha-1-antichymotrypsin in both portions support that histologically different components of this neoplasm derive from the same origin. To our knowledge, this is the first case of dedifferentiated chordoma in Korea.
Male ; Humans