Plasmacytoid Myoepithelioma of the Palate: Case Report

Myoepitheliomas are extremely rare benign neoplasms of salivary glands. They represent 1–1.5% of all salivary gland tumors. They occur, principally, in the parotid gland and infrequently in minor salivary glands. There are distinct histological and immunohistochemical characteristics of the tumor which aid in the diagnosis. Myoepithelioma of the palate is uncommon and only a limited number of cases has been reported in the English literature. Herein, a case of plasmacytoid type of myoepithelioma of the palate in an adult female is presented along with review of the literature.     

Malignant Mesothelioma of the Epididymis: A Case Report

Malignant mesothelioma of the epididymis is a very rare tumor. We report a case of a 33-year old male with a left hydrocele that presented for evaluation of infertility. Scrotal ultrasound revealed a left extratesticular mass. Final pathology revealed malignant mesothelioma involving the epididymis, with well differentiated epithelioid morphology. Metastatic workup was negative.Key Words: Epididymis, Malignant mesothelioma     

Malignant Peritoneal Mesothelioma: a Case Report

In this paper we report a case of malignant peritoneal mesothelioma, a rare abdominal tumour. A 72-year-old male with a medical history of heart disease presented to our Clinic because of pain in the right half of the abdomen. Diagnostic procedures, including clinical and laboratory examination, X-ray, ultrasonography and computed tomography, revealed a tumour in the right lower quadrant of the abdomen. The approximate size of the tumour size at initial detection was 7 cm. During the pre-operative procedure an evident growth of the tumour was noticed, indicating exploratory laparotomy. Intra-operative findings revealed a large tumour of the anterolateral abdominal wall, involving the greater omentum. Tumour resection was performed, as well as resection of the portion of the anterolateral abdominal wall and omentectomy. Postoperative immunohistochemical analysis revealed malignant peritoneal mesothelioma.     

前列腺恶性纤维组织细胞瘤1例报告

目的 :探讨前列腺恶性纤维组织细胞瘤 (MFH)的诊断和治疗。　方法 :诊治 1例前列腺MFH病人。行前列腺肿瘤根治术 +直肠切除术 +乙状结肠造瘘术。并结合文献进行诊治讨论。　结果 :术后 3周复发 ,遂行放疗 ,2个月后因肿瘤转移而死亡。　结论 :前列腺MFH预后差 ,早期原发病灶切除并术后辅助治疗是关键     

Giant Malignant Cystic Pheochromocytoma: A Case Report

Cystic malignant pheochromocytomas are uncommon. Differing from solid pheochromocytomas, which produce catecholamines and present adrenergic syndrome, cystic pheochromocytomas, may not produce these. Preoperative diagnosis may be difficult. Ct scan is useful for preoperative management. In this report, we describe a case of a giant malignant cystic pheochromocytoma in a young woman (17 years old) which presented as giant abdominal mass. The malignancy was confirmed by the presence of liver metastasis. Two years after curative resection, the patient is in good health with no recurrence.     

Amelanotic Malignant Melanoma of the Rectum: Report of a Case

We describe herein a case of amelanotic melanoma of the rectum. Macroscopically, the tumor was lacking in pigmentation and microscopically, it was found to be deficient in melanin. Moreover, the tumor cells showed positive staining for HMB-45, the antimelanoma antibody, which led to a diagnosis of amelanotic melanoma. The patient died of metastatic tumors in the liver and bone 17 months after undergoing abdominoperineal resection of the rectum and dissection of the bilateral inguinal lymph nodes. Received: June 12, 2000 / Accepted: January 9, 2001     

Malignant Sarcomatoid Tumor of the Liver: Report of a Case

A 65-year-old man was referred to our hospital for treatment of a liver tumor. Abdominal ultrasonography (US) demonstrated a low echoic mass in the S2–S4 region of the liver, which was confirmed by abdominal computed tomography (CT). In the delayed phase of angio-CT, the inside of the mass was not enhanced. Abdominal angiography showed a hypovascular area in the liver. An extended left lobectomy was performed. Macroscopically, the tumor was 9.5 × 9.5 cm in size, and on cross section, it was white and clearly demarcated from the surrounding tissue. Microscopic observation of hematoxylin–eosin-stained specimens did not show any glandular or trabecular formation. Histologically, there was diffuse proliferation of atypical spindle cells that had hyperchromatic, short, spindle-shaped nuclei, and pale cytoplasm with poor intercellular adhesion. The nontumorous tissue was almost normal with no sign of cirrhosis. Immunohistochemical examination showed that the spindle cells were positive for vimentin and cytokeratins (AE1/AE3, CAM 5.2), but negative for all other markers. The final diagnosis was a sarcomatoid carcinoma, the origin of which was not able to be confirmed immunohistochemically. This case of a primary hepatic tumor composed of malignant cells with sarcomatous features is described, and the immunohistochemical findings are discussed. Received: February 21, 2000 / Accepted: July 25, 2000     

Primary Malignant Melanoma of the Small Intestine: a Case Report

The small intestine is the most common site of gastro-intestinal metastasis from cutaneous malignant melanoma. A primary origin at this site has been reported in rare cases. We report a case of a 71-year-old man with a primary malignant melanoma in the jejunum. The patient presented with weakness, weight loss, non-specific abdominal pain and episodes of fainting. After clinical examination, laboratory evaluation and radiological work-up, which included CT of the abdomen, the patient was diagnosed with a tumour mass in the jejunum. This diagnosis was confirmed at laparotomy. The patient underwent enterectomy with wide excision of the tumour. A primary malignant melanoma of the small intestine is an extremely rare neoplasm.

A definite diagnosis can only be made after a thorough investigation has been made to exclude the co-existence of a primary lesion elsewhere. Curative resection of the tumour remains the treatment of choice.     

Malignant Granular Cell Tumor of the Esophagus: A Case Report

Introduction: Malignant granular cell tumor (MGCT) of the esophagus is an extremely rare malignancy with a poor prognosis. Literature describing this condition is not sufficient, especially regarding long-term survival.Presentation of Case: A 52-year-old woman presented with dyspnea and slow onset dysphagia. The endoscopy, endoscopic ultrasound (EUS), bronchoscopy, and positron emission tomography (PET)/computed tomography (CT) supported the suspicion of esophageal gastrointestinal stromal tumor (GIST). Open wedge esophagectomy and tracheal resection were performed. The histology proved periodic acid–Schiff (PAS)-positive granules in epithelial cells, hyperchromatic nuclei and the positivity of Protein soluble in 100% ammonium sulfate (S-100), vimentin, neuron-specific enolase, laminin, and myelinic proteins. Local recurrence after 10 months required a two-phase esophagectomy with retrosternal gastroplasty. Bone, liver, and mediastinal metastases occurred 6 months later, with overall survival of 34 months.Discussion: Preoperative histological confirmation is often not reliable. Tracheal invasion increases the perioperative risk and the probability of an unsuccessful resection. Esophagectomy or radical R0 local resection is the only known curative therapy. Repeated resections may increase survival in case of locoregional recurrence. Radiotherapy has a potential for palliative care.Conclusion: Esophageal MGCT requires a detailed presentation including long-term survival. Early surgical removal of intramural esophageal neoplasms with potentially malignant features is highly recommended. Radical and/or repeated esophageal resections are the only known therapies with curative potential.     

Ileal Malignant Melanoma Causing Intussusception: Report of a Case

Cutaneous malignant melanoma (MM) often metastasizes to the gastrointestinal (GI) tract; however, primary MM of the small intestine is a controversial diagnosis. We report the case of a 76-year-old woman found to have a primary MM in the ileum. After clinical evaluation, the radiological workup, which included magnetic resonance enteroclysis (MRE), revealed a large polypoid intraluminal tumor. She underwent laparotomy and the lesion was excised. Histological examination of the resected specimen revealed morphological and immunohistochemical characteristics of MM and a detailed postoperative examination failed to identify a primary lesion on the skin, anus, oculus, or any other site. The patient died of brain metastasis 6 months after surgery. According to our review of the literature, this is the first case of primary MM of the small intestine diagnosed with the help of MRE.     

Myxoid Malignant Fibrous Histiocytoma of the Breast: Report of a Case

Malignant fibrous histiocytoma (MFH) is the most common type of soft tissue sarcoma, but it rarely develops as a primary tumor in the breast. Furthermore, no case of the myxoid variant of MFH in the breast has ever been documented. We report the case of a 52-year-old woman with a breast tumor that was immunohistochemically confirmed to be myxoid MFH. She underwent a radical mastectomy and is currently well with no evidence of local recurrence or metastatic spread after 3 years of follow-up. Received: August 6, 2001 / Accepted: March 5, 2002     

Primary Malignant Melanoma of the Small Intestine: Report of a Case

The small intestine is the most common site of gastrointestinal (GI) metastases from cutaneous malignant melanoma; however, primary malignant melanoma originating in the small intestine is extremely rare. We report the case of a 72-year-old man found to have a primary malignant melanoma in the ileum. The patient presented with anorexia, weight loss, diffuse colicky abdominal pain, and episodic rectal bleeding. A preoperative diagnosis of a small intestinal tumor was based on the findings of enteroclysis and computed tomography scanning. This diagnosis was confirmed at laparotomy and an enterectomy was performed. Histopathological examination of the resected specimen clarified the exact nature of the lesion, confirming the diagnosis of melanoma. A thorough postoperative investigation did not reveal a primary lesion in the skin, anus, oculus, or any other location. Thus, we diagnosed this tumor as a primary lesion. One year after his operation, the patient remains well without any evidence of recurrence. Primary malignant melanoma of the small intestine is an extremely rare lesion, which must be differentiated from other intestinal tumors. Received: August 6, 2001 / Accepted: March 5, 2002     

Inflammatory Malignant Fibrous Histiocytoma of the Gallbladder: Report of a Case

We describe herein a case of inflammatory malignant fibrous histiocytoma (IMFH) of the gallbladder that subsequently metastasized to the ascending colon and later to the stomach. A 70-year-old Japanese man with a palpable mass in the right upper quadrant of the abdomen was referred to our hospital for investigation and treatment. Laboratory data showed severe leukocytosis and elevated serum granulocyte colony-stimulating factor (G-CSF) concentrations. A laparotomy was performed, and the tumor was excised en bloc with the gallbladder and part of the liver bed. Histopathologically, the tumor was composed of ordinary malignant fibrous histiocytoma (MFH) components characterized by pleomorphic tumor cells, bizarre giant cells, and conventional spindle cells in a storiform growth pattern, as well as a xanthogranulomatous component, including inflammatory cells, foamy histiocytes, and plasma cells. Immunohistochemical study revealed that the pleomorphic tumor cells and bizarre giant cells were positive for antibodies against α₁-antitrypsin and α₁-antichymotrypsin. The final pathologic diagnosis was IMFH. The tumor cells were diffusely positive for anti-G-CSF monoclonal antibody, and the inflammatory reaction subsided immediately after tumor resection, strongly suggesting that the primary tumor cells produced G-CSF. This patient is still alive with no signs of recurrence more than 3 years after his primary operation, which to our knowledge is the longest survival period ever reported. Therefore, visceral IMFH is manageable in some cases by resecting the primary and isolated metastatic lesions. Received: October 11, 2000 / Accepted: May 15, 2001     

Malignant Fibrous Histiocytoma of the Abdominal Cavity: Report of a Case

Malignant fibrous histiocytoma (MFH) is a soft-tissue sarcoma originating from fibroblast cells, characterized by a high rate of metastasis or recurrence. This tumor rarely develops in the gastrointestinal tract, with no more than 30 cases described in the literature. We report a case of MFH of the abdominal cavity in a 45-year-old woman who presented with epigastric pain, anorexia, and weight loss. A computed tomography (CT) scan of the abdomen revealed multiple solid tumors in the peritoneal cavity. We performed exploratory laparotomy and found at least 15 solid whitish tumors attached to the wall of the small intestine, as well as to the parietal peritoneum. There were three metastases in the liver. All of the tumors were excised, most of which were about 10cm in diameter. Histopathological findings indicated a stromal tumor consisting of spindle cells, and immunohistochemical examination of the resected specimens established the definite diagnosis of a pleomorphic MFH. The patient had an uneventful postoperative course and was given adjuvant chemotherapy. She is currently well 2 years after her operation. We review the clinical picture of this tumor in the abdominal cavity, and discuss its diagnosis, pathogenesis, and treatment.     

Gastrointestinal Metastases from Malignant Melanoma: Report of a Case

Malignant melanoma metastases in the gastrointestinal tract (GIT) are found in more than 60% of autopsies on patients who have died with disseminated melanoma; however, the rate of GIT metastases detected clinically averages only 2%. This discrepancy seems to be attributed to the nonspecific symptoms and signs of GIT involvement, which include weakness, fatigue, bleeding, anemia, and abdominal pain. Sometimes a diagnosis is only made when bowel obstruction occurs. We report a case of long-term survival after surgery for multiple melanoma metastases in the gastrointestinal tract and review the relevant literature. Both our case report and the literature review demonstrate the benefits of surgery for patients with melanoma metastases in the GIT. We also stress the need for meticulous follow-up, detailed history-taking, and rapid evaluation of any vague and unclear abdominal signs and symptoms for patients with melanoma.     

Primary Malignant Mesothelioma of the Greater Omentum: Report of a Case

We report a rare case of primary malignant mesothelioma of the greater omentum. To our knowledge, only one other such case has been described in the English literature. The patient was a 61-year-old Taiwanese woman without any history of exposure to asbestos, who presented with lower back pain. Abdominal sonography and computed tomography showed a 12 × 9 × 9-cm³ mass occupying the lower abdomen. Laparotomy revealed a tumor in the greater omentum, invading the posterior wall of the uterus, without diffuse mesenteric thickening or multiple small nodules in the peritoneum. We performed en bloc resection of the mass, which involved omentectomy, hysterectomy, and bilateral salpingo-oophorectomy. Microscopically, the tumor cells were arranged in a tubulopapillary pattern lined by a single layer of uniform, cuboidal cells. A pattern of sclerotic stroma with irregular glandular elements was also recognized. Immunohistochemically, the tumor cells showed strong positivity for calretinin. The final pathologic diagnosis was malignant mesothelioma. The patient did not receive chemotherapy or radiotherapy, and has remained in good health without any evidence of recurrence for almost 3 years since her operation.     

Malignant Eccrine Spiradenoma: A Case Report and Review of the Literature

BACKGROUND: Eccrine spiradenoma is a well-differentiated benign tumor of the sweat glands. Malignant change arising within eccrine spiradenoma is rare. OBJECTIVE: We describe a patient with malignant eccrine spiradenoma exhibiting both carcinomatous and sarcomatous differentiation. METHODS: Case report and literature review. RESULTS: A 37-year-old woman noted enlargement of a left axillary tumor that had been present for 20 years. The tumor was resected and the specimen, measuring 3.0 cm x 1.5 cm, revealed an encapsulated benign eccrine spiradenoma as well as an undifferentiated carcinoma possessing both carcinomatous and sarcomatous components. A transition zone was evident between the benign eccrine spiradenoma and the undifferentiated carcinoma, suggesting that the latter had arisen from the benign tumor. The malignant areas consisted principally of undifferentiated carcinoma (70%), although squamous cell carcinoma (10%), adenocarcinoma (10%), and chondrosarcomatous (10%) components were also present. Numerous mitotic figures were noted within the areas of malignant change, suggesting that the tumor was aggressive in nature. The patient died of systemic metastases 7 months after diagnosis. CONCLUSION: Although eccrine spiradenomas are usually benign, they can, on rare occasions, undergo malignant transformation. This case report describes one such occurrence of malignant transformation of a benign eccrine spiradenoma that unfortunately resulted in the patient's death from systemic metastases 7 months after diagnosis.     

Malignant Glomus Tumor: A Case Report and Review of the Literature

Glomangiosarcoma, or malignant glomus tumor, is a very rare neoplasm that when seen typically arises from a benign glomus tumor. Despite having histologic features of malignancy, these tumors usually do not metastasize. However, when metastasis occurs this disease is often fatal. We report a case of a malignant glomus tumor arising de novo on the nose of an 89-year-old white woman, and we review the literature concerning glomangiosarcomas.     

Malignant Fibrous Tumor of the Pleura: Case Report and Literature Review

Solitary fibrous tumours (SFT) of the pleura are uncommon and are incidental findings or discovered in patients with non-specific respiratory symptoms. We report a case of a 74 year old man diagnosed with a mesenchymal pleural neoplasm, associated with typical hypertrophic osteoarthropathy, referred to as Pierre-Marie-Bamberg syndrome. As reported in the literature, complete surgical resection is the gold standard for treatment of such lesions and recurrences. Radiotherapy and chemotherapy are of limited value in the curative treatment of pleural SFT. In our case surgical excision of the mass was performed. After a disease-free period of 3 years a second intervention was necessary because of recurrence. Until now our patient is free of complaints and no signs of reappearance were noted. Based on our experience and on literature findings we would like to underline the importance of regular long-term follow-up because of the substantial risk of recurrence.