Lymphangiomyomatosis: A report of three cases treated with tamoxifen

Summary Three cases are reported of lymphangiomyomatosis with pulmonary and abdominal manifestations. Two had a chylous pleura effusion, while the third presented a retroperitoneal manifestation, which was completely resectable. Antiestrogen therapy with tamoxifen was administered in all three cases. Two patients died of pulmonary progression after 4 months of therapy. The third is still alive, with stable disease for more than 6 years, and has been receiving tamoxifen for 66 months. These observations indicate that antiestrogen treatment may be as effective as oophorectomy when started at an early stage of the disease.     

Extra-gastrointestinal stromal tumor of the pelvic cavity: case report

Extra-gastrointestinal stromal tumors (E-GISTs) not associated with the alimentary tract in the pelvic cavity are extremely rare. We treated a 49-year-old Japanese man with such an E-GIST in the pelvic cavity who underwent an intrapelvic tumorectomy with a total prostatectomy and partial rectum resection. Gross examination of the specimen revealed an 8.1 × 5 × 4 cm white-grayish mass. Histological findings showed uniform spindle cells with scant atypia that formed interlacing bundles or whorl patterns. These neoplastic cells did not invade adjacent organs, including the gut. Immunohistochemical findings revealed that the neoplastic cells were positive for c-kit, CD34, and vimentin. Molecular analysis showed a c-kit mutation at exon 9 with duplication of Ala and Tyr. Our diagnosis was E-GIST, which belongs to the intermediate group of GIST. Following the operation, we administered imatinib mesylate for 6 months. After stopping for 5 months, it was administered again for local recurrence. We are planning our future strategy for this case including surgical resection as necessary.     

Squamous cell carcinoma arising within verrucous carcinoma of the oral cavity: a case report

The author herein reports a case of squamous cell carcinoma (SCC) arising within verrucous carcinoma (VC) of the hard palate. An 84-year-old woman was admitted to our hospital complaining of oral discomfort. Oral examination revealed a pedunculated verrucous tumor (15 x 15 mm) in the hard palate. A biopsy revealed verrucous tumor. Resection of the lesion with wide margins was performed. Grossly, the palate tumor was pedunculated and verrucous, but a depressed area (8 x 7 mm) was recognized. Microscopically, the verrucous ares showed verrucous proliferation of squamous epithelium with little cellular atypia, and was interpreted as VC without invasion. The depressed lesion was obvious SCC with invasion. There were direct transitions between the VC and SCC. Immunohistochemically, the VC and SCC tumor cells were negative for human papilloma virus antigens. P53 protein was expressed in both VC and SCC, though the expression in SCC was much more strong and broad than that in VC. The Ki-67 antigen was also expressed in the VC and SCC, and Ki-67 labeling index ranged was 12% in VC and 64% in SCC. These findings indicate that SCC may arise within VC.     

Epithelial-myoepithelial carcinoma arising in the nasal cavity: a case report and review of literature.

Epithelial-myoepithelial carcinoma is an uncommon, low-grade, malignant epithelial neoplasm composed of variable proportions of ductular cells and large, clear staining, myoepithelial cells arranged around the periphery of the ducts. About 120 cases have been reported in the world literature, most of which were located in salivary glands, except for a few cases occurring in unusual locations such as breast, lacrimal gland, nose, paranasal sinus, trachea, bronchus, and lung. We here reported the second case of epithelial-myoepithelial carcinoma of the nasal cavity with extension to the nasopharynx. The patient was a 61 year old Chinese female with two month's history of progressive nasal obstruction. Histopathologically, the tumor showed typical myoepithelial and ductal cells biphasic differentiation, duct-like structure and infiltrating growth pattern. Some ductal cells showed the characteristics of oxyphilic cell, which had never been reported before. Recurrence and metastasis rates of epithelial-myoepithelial carcinoma varied from 35% to 50% and 8.1% to 25% respectively in different reports. The present case had neither recurrence nor metastasis twenty months after operation. When epithelial-myoepithelial carcinoma is mainly composed of spindle myoepithelial cells, the differential diagnosis should include myoepithelioma, neurofibroma, leiomyoma and hemangiopericytoma.     

Hemangioblastoma of pelvic cavity: report of a case and review of literature

Hemangioblastoma of soft tissue is a very rare tumor of uncertain histological type. Herein, we reported a 51-year-old woman was found to have a solid and cystic mass measuring 31×30 mm in the right adnexa area on a computed tomography scan. The tumor showed the typical histology of hemangioblastoma. Tumor was composed of numerous capillaries and stromal cells with cytoplasmic vacuolization. Immunohistochemical study revealed that the tumor stromal cells were positive for CD56, S-100 protein, NSE, Syn, CgA, and inhibin-α. Focal EMA positivity was present. Ki-67 expression was found in approximately 1% of tumor cells. The tumor cells were negative for CK, HMB-45, Melan-A, SMA, and CD68. The differential diagnosis of Hemangioblastoma arising in pelvic cavity includes hemangioma, hemangioendothelioma, liposarcoma, renal cell carcinoma, fat-forming solitary fibrous tumor, paraganglioma, and perivascular epithelioid cell tumor (PEComa).     

Lymphadenoma arising in the parotid gland: a case report

We report a case of lymphadenoma arising in the parotid gland. A 53-year-old female patient presented with a mass in the parotid gland. Grossly, it was a well-demarcated solid mass measuring 3 cm in diameter. Microscopic examination revealed many cysts or duct-like structures in the background of the prominent lymphoid stroma, confirming a diagnosis of lymphadenoma. This particular case was thought to have arisen from an intraparotid lymph node. Lymphadenoma is a rare benign neoplasm of the salivary gland with partial resemblance to other salivary gland tumors, such as Warthin's tumor, cystadenoma, sebaceous lymphadenoma or mucoepidermoid carcinoma. Therefore proper recognition of this rare entity is warranted to avoid confusion in the diagnosis.     

与血管平滑肌瘤伴发的血管内淋巴瘤病一例

患者男,54岁.因反复寒战、发热3个月于2002年10月5日入院.患者在无明显诱因下先出现寒战,0.5 h后全身发热,体温最高达40.4℃,1.5 h后体温降至正常,反复发作,每天2～3次.     

Neurocytoma arising in the pelvis

Central neurocytoma represents a rare neoplasm of the central nervous system with advanced neurocytic and sometimes focal lipomatous differentiation, a low proliferative potential and a favorable prognosis depending on the efficiency of surgical resection. This entity has been described as an intraventricular tumor near the foramen Monroi. Here, we report a case of a 21-year-old male with peripheral neurocytoma. Using computed tomography, a tumor of unknown origin was located behind the bladder. After complete surgical resection of the tumor, histologically small uniform cells, zones of fibrillarity and neuropil-like islands were seen. Immunohistochemistry revealed positivity for the neuronal markers synaptophysin, neuron-specific enolase and neurofilaments. Vimentin, pan-keratin, desmin, chromogranin, CD-99 and glial fibrillary acidic protein were immuno-negative. A low proliferation rate (1–2%) was found. Several case reports described extraventricular central neurocytomas. A sole publication documented a peripheral neurocytoma arising within a mature cystic teratoma of the ovary. To our knowledge, this is the second reported case of a neurocytoma outside the central nervous system, indicating that this entity may also occur infrequently in peripheral tissues.     

Reactive nodular fibrous pseudotumor involving the pelvic and abdominal cavity: a case report and review of literature

There are several entities that can present with multiple nodular lesions within the peritoneal cavity, such as “disseminated peritoneal leiomyomatosis,” “peritoneal fibrosis,” “calcifying fibrous pseudotumor,” and the recently described lesion of “reactive nodular fibrous pseudotumor of the gastrointestinal tract and mesentery.” Here we present one such lesion in a 28-year-old woman with a history of dysmenorrhea and ergotamine use for migraine attacks. Intraoperative exploration of our patient disclosed numerous nodules located throughout the pelvic and abdominal peritoneum. Histopathologically, these lesions were fibrocollagenous nodules composed of sparse wavy spindle cells within hyalinized “keloid-like” collagen surrounded by an inflammatory infiltrate. Some of the nodules were associated with small foci of endometriosis. We believe this lesion is best described by the term “reactive nodular fibrous pseudotumor” and that endometriosis and the use of ergotamine derivatives may be contributing factors.     

Mucoepidermoid carcinoma arising in Warthin's tumor: a case report

Malignant transformation of the epithelial component of Warthin's tumor is extremely rare. We describe our experience of mucoepidermoid carcinoma arising in Warthin's tumor of the parotid gland in a 35 year old female. The tumor removed from the parotid region was well encapsulated and histologically comprised ofmucoepidermoid carcinoma along with areas of Warthin's tumor. The pathogenesis and differential diagnosis of this rare occurrence have been discussed.     

Adenocarcinoma arising in gastric heterotopic pancreas: a case report

A heterotopic pancreas in the gastrointestinal tract is mostly found incidentally and its malignant transformation is extremely rare. We describe the second case of adenocarcinoma arising in a gastric heterotopic pancreas of an asymptomatic 35-yr-old man in Korea. Esophagogastroduodenoscopy revealed a submucosal tumor with an irregular central umbilication in the gastric antrum. A wedge resection specimen demonstrated a submucosal oligolocular cystic mass (1.7 x 1.4 x 1.2 cm) with a solid portion. Microscopically, the cystic portion was composed of dilated pancreaticobiliary type ducts with adjacent small foci of periductal glandular structures. The adenocarcinoma components in the solid area infiltrated the proper muscle and the overlying mucosa of the stomach. The transitional area between the benign ductal structures and the adenocarcinoma component was found. The follow-up course was uneventful 5 months postoperatively.     

Mammary carcinoma arising in microglandular adenosis: a case report

Microglandular adenosis is an uncommon benign breast entity considered as a variant form of adenosis. The diagnosis is frequently made by the pathologist as it is often clinically asymptomatic. Carcinoma arising in microglandular adenosis is very uncommon. We report a case of carcinoma arising in microglandular adenosis in a 54-year-old woman. The immunohistochemical profile, especially S100 protein expression and absence of epithelial membrane antigen, was useful to recognize the microglandular adenosis and the carcinoma of alveolar architecture, while invasion was suggested by the basement membrane disruption highlighted with anti-collagen IV immunostaining. The good prognosis of carcinoma associated with microglandular adenosis points out the importance of distinguishing this lesion from other breast carcinomas.     

Chordoma arising in a mature cystic teratoma of the ovary: a case report

Mature cystic teratoma of the ovary (MCTO) is the most common type of ovarian teratoma and also the most frequent tumor originating from germ cells. It is usually diagnosed in early adulthood and, by definition, is composed of well-differentiated tissues, which originate from all three germ cell layers. Unusual types of tissues can be found in MCTO, such as kidney, adrenal, and prostatic tissues. Malignant transformation is reported in less than 2% of teratomas. Squamous cell carcinoma is the most common malignancy arising in these otherwise benign tumors. We present the first case of MCTO containing a chordoma. The chordoma differentiation was supported by immunohistochemical staining and interphase fluorescence in situ hybridization (IP-FISH) technique showing 19% of the nuclei of the MCTO displaying polysomy for the chromosome X, while 28% of the chordoma nuclei showed chromosome 7 mosaicism. These results are concordant with previous studies, showing chromosomal anomalies in chromosomes X and 7 in MCTO and chordomas, respectively.     

Leiomyosarcoma arising from tunica vaginalis testis: a case report

Benign and malignant soft tissue tumors of the paratesticular region i.e. those arising from the testicular tunics, epididymis and spermatic cord are uncommon. Of these, leiomyosarcoma arising from the tunica vaginalis is extremely rare. On extensive computerised search, a single case has been reported till date in the literature. We hereby report one such case because of its rarity.     

Pigmented mucoepidermoid carcinoma of the oral cavity: a case report

Melanocytes are normally present in the oral mucosa, but unlike the skin, melanocytic colonization and pigmentation of nonmelanocytic tumors rarely occur. A case of pigmented mucoepidermoid carcinoma (MEC) in a 36-year-old woman is reported. On H&E-stained sections, brownish granules were seen in intermediate and epidermoid neoplastic elements and in elongated or dendritic cells showing histologic and immunohistochemical features of melanocytes. Ultrastructurally numerous epithelial cells contained large aggregates of melanin granules, a feature suggestive of phagocytosis. In addition small individual melanin granules were observed at the external border of the epithelial cell membrane. These features suggest that melanocytes colonized the MEC and that neoplastic cells may phagocytose the granules, similarly to what occurs with epidermal melanocytes. The phenomenon of melanocyte colonization of nonmelanocytic tumors should be taken into consideration in dealing with pigmented lesions of the oral cavity.     

Choriocarcinoma arising in transitional cell carcinoma of bladder: a case report

Trophoblastic metaplasia can occasionally occur in transitional cell carcinomas of the bladder and the resultant choriocarcinoma is likely to behave differently from typical epithelial bladder tumours.     

腹壁瘢痕子宫内膜异位症恶变为腺肉瘤一例

患者女,26岁.G0P0,痛经多年.患者因直肠息肉切除术后切口疼痛、渗血加剧于2006年9月来本院就诊.患者6年前因反复腹泻伴便血曾在当地医院就医.     

Intestinal adenocarcinoma arising in a mature cystic teratoma of the ovary: a case report

Gastrointestinal adenocarcinoma arising in mature cystic teratomas of the ovary is extremely rare. We report a case of well-differentiated intestinal adenocarcinoma arising in a mature cystic teratoma of the ovary in a 77-year-old woman, presenting as acute abdomen with ovarian torsion. An immunohistochemical study revealed expression of CK20 and CK7, and the tumor was also positive for MUC2. The patient had no evidence of disease after 12 months of follow-up.