Primary adenocarcinoma of the epididymis: case report

Primary epididymal malignancies are uncommon and usually benign. Benign paratesticular tumors are most commonly adenomatoid, while the most common malignant paratesticular tumors are rhabdomyosarcomas. Approximately 25% of all epididymal tumors are malignant, and of the benign tumors, 60% to 78% are adenomatoid tumors. According to a recent MEDLINE search using epididymis and adenocarcinoma as key words, reports of a primary epididymal adenocarcinoma are extremely rare with only 23 cases in the literature. We report a case of epididymal adenocarcinoma with clinical follow up and metastatic natural history of this rare malignancy.     

Primary adenocarcinoma of the epididymis: Case report

Primary epididymal malignancies are uncommon and usually benign. Benign paratesticular tumors are most commonly adenomatoid, while the most common malignant paratesticular tumors are rhabdomyosarcomas. Approximately 25% of all epididymal tumors are malignant, and of the benign tumors, 60% to 78% are adenomatoid tumors. According to a recent MEDLINE search using epididymis and adenocarcinoma as key words, reports of a primary epididymal adenocarcinoma are extremely rare with only 23 cases in the literature. We report a case of epididymal adenocarcinoma with clinical follow up and metastatic natural history of this rare malignancy. This revised version was published online in August 2006 with corrections to the Cover Date.     

Primary Merkel Cell Carcinoma of the Submandibular Gland: When CK20 Status Complicates the Diagnosis

Merkel cell carcinoma is a neuroendocrine tumor that occurs predominantly on the sun-exposed skin, with rare cases in the extracutaneous sites. It represents one of the extremely rare malignant neuroendocrine tumors of the salivary glands. We report a case of primary Merkel cell carcinoma of the right submandibular gland. The preoperative diagnosis was doubtful and the definitive histological diagnosis proved to be very difficult considering the extreme rarity of this tumor. The intraoperative evaluation of the macroscopic characteristics of the lesion led to an elective lymph node dissection. The extreme aggressiveness of the disease has resulted in the necessity of a new post-operative staging and in a multimodal treatment. This is the first primary submandibular gland Merkel cell carcinoma described in the literature. Differential diagnosis may be challenging and proper hematoxylin-eosin staining and immunohistochemical studies are mandatory.     

A case of metastatic renal tumor originating from a malignant mixed tumor of the submandibular gland]

A rare case of metastatic renal tumor originating from a malignant mixed tumor of the submandibular gland is reported. The patient was a 45-year-old woman with hard masses palpated in the right upper arm and the right upper gluteal region. She had a history of surgical resection of a right submandibular tumor followed by radiotherapy in 1977. Biopsy findings of the gluteal mass suggested a malignant clear cell tumor. A chest X-ray, excretory urogram, CT scan, MRI and renal angiogram were highly suggestive of a right renal cell carcinoma with metastases to the right lung (S1 & S10), the left 4th and 5th ribs, the right upper arm muscle and the right upper gluteal muscle. Right nephrectomy and resection of the right upper arm and right upper gluteal tumors were performed. Microscopic examination showed that all tumors were malignant mixed tumors. When compared to the previous surgical specimen, the tumors were believed to be identical. The tumors of the lung and ribs subsequently resected were malignant mixed tumors. The patient is well with no objective or subjective evidence of recurrent neoplastic disease 7 months post-operatively. To our knowledge only one report of metastatic renal tumor originating from a malignant mixed tumor of the salivary gland has been published in Japan. But metastatic renal tumor originating from the same tumor of the submandibular gland has not been reported previously in Japan.     

Schwannoma of the submandibular gland

Neurilemmomas (schwannomas) are solitary neurogenic tumors that arise from cells of the neural sheath. They are slow growing and represent a proliferation of Schwann cells. Extracranial neurogenic tumors of the head and neck are uncommon, with benign tumors occurring with greater frequency than malignant tumors. These tumors often mimic primary or metastatic disease in the head and neck. Schwannoma of the salivary gland is a particularly rare form of an extracranial neurogenic tumor, with most presenting in the parotid gland originating from a peripheral branch of the facial nerve. An unusual case of neurilemmoma of the submandibular gland is presented, and the literature concerning this subject is reviewed. We believe this tumor originated in an autonomic nerve of the submandibular gland. The mass was discrete and well demonstrated on CT scan. Total excision of the gland resulted in complete resolution of symptoms with no cranial nerve deficits.     

Recurrent primary cardiac osteosarcoma: a case report and literature review

Primary malignant cardiac tumors are very rare. Among malignant tumors, sarcomas occupy first place. In particular, primary cardiac osteosarcoma is extremely rare. To the best of our knowledge, only 42 cases have been reported worldwide. Cardiac malignant tumors usually require complex operations due to the difficulty in completely removing the tumor with acceptable free surgical margins and because of the proximity to vital structures. The current multimodality treatment strategies for cardiac sarcoma are still suboptimal, and surgery in particular frequently has unsatisfactory results. We report a case of recurrent primary cardiac osteosarcoma in a young male who underwent trans-sternal right pneumonectomy and a wide resection of the left and right atrium followed by reconstruction with heterologous pericardium under extracorporeal circulation. The patient died 6 months after the operation due to local and systemic disease recurrence.     

Retroperitoneal rhabdomyosarcoma revealed by acute kidney failure in children. Report of a case

Retroperitoneal sarcomas are rare tumors representing 0.1% to 0.2% of all malignant tumors. They are usually revealed by an abdominal mass (33%), possibly associated with pain (41%). Edema of the lower limbs is reported in 10% of cases. Acute renal failure with anuria by bilateral ureteral compression, associated with invasion of the inferior vena cava has not been previously reported.     

Hepatobiliary cystoadenomas

Cystoadenomas of the liver are rare cystic tumors that have seldom been reported. Accurate preoperative diagnosis is difficult because they are usually mistaken for more frequent lesions. Due to their malignant potential, complete surgical removal of the lesion is required. Correct diagnosis is guided by clinical history, radiological imaging, and laboratory parameters. We report two of these rare cases and discuss the spectrum of presentation, pathological features and treatment of these tumors.     

Solitary submandibular soft tissue osteochondroma: A rare case report

IntroductionSoft tissue osteochondromas are rare benign tumors containing bone and cartilage that form in mesenchymal tissues with no connection to adjacent bone, cartilage, or periosteum. They mimic endochondral ossification and are usually encased in a fibrous tissue capsule. The occurrence of the tumor is extremely rare in the maxillofacial region.Case presentationA case of soft tissue osteochondroma in the submandibular region of a 47-year-old man with a medical history of muscular dystrophy and schizophrenia is presented here. The tumor had been gradually growing for 11 years before the patient's presentation to our clinics. Radiographic and clinical examination revealed a radiopaque mass in soft tissue that extended from the left anteroinferior border of the mandible and with no connection to the mandibular periosteum. Treatment involved surgical excision. A diagnosis of soft tissue osteochondroma was established by histopathological examination.Clinical discussionPreviously, there have been only three reported cases of soft tissue osteochondroma in the maxillofacial region. The cause of this tumor is not clearly understood, with multiple hypotheses being proposed. Diagnosis usually involves radiology and histopathology, and the tumor is completely amenable to surgical excision.ConclusionExtraskeletal osteochondroma should be considered in the differential diagnosis of hard tissue lesions with no apparent connection to the underlying bone in the maxillofacial region. Although considered rare, clinical awareness about this tumor aids the practitioner in identifying, diagnosing and properly managing this tumor. There has been no report of recurrence or malignant transformation of the lesion.     

不同病理类型并存的甲状腺恶性肿瘤临床分析

目的 探讨不同病理类型并存的甲状腺恶性肿瘤的临床诊断、治疗方法和预后.方法 对1977年1月-2006年12月收治的7例不同病理类型并存的甲状腺恶性肿瘤病例进行回顾性分析.结果 不同病理类型并存的甲状腺恶性肿瘤占本院同期甲状腺恶性肿瘤的0.14%,影像学及实验室检查均无特异性诊断价值,不同病理类型并存的甲状腺癌采用甲状腺癌根治手术配合内分泌治疗及[3]I治疗可取得满意疗效,甲状腺癌合并甲状腺其他恶性肿瘤预后差. 结论不同病理类型并存的甲状腺恶性肿瘤属于罕见疾病,发病机制尚不明确,命名也不统一,目前尚无特异性诊断方法,其预后取决于恶性程度最高的病理类型.     

Primary malignant triton tumor of the foot

. The malignant Triton tumor is a relatively rare, usually aggressive sarcoma comprised of both malignant schwannoma cells and malignant rhabdomyoblasts. Histopathologically the tumor is described as malignant with neurogenic and myogenic differentiation. A review of all reported cases of Triton tumors in the literature calls attention to their tendency to occur in neurofibromatosis. In this report we present a malignant Triton tumor of the foot not associated with von Recklinghausen's neurofibromatosis.     

Evolution and outcome in malignant astroglial neoplasms of the cerebellum

Malignant astroglial neoplasms of the cerebellum are rare and the clinical behavior of these tumors is unpredictable. The authors describe the histological characteristics and clinical outcome in 10 patients; eight had malignant cerebellar astrocytomas, and one each had an astroblastoma and a true polar spongioblastoma. Malignant glial tumors involving the cerebellum usually behave very aggressively. Cerebellar astroblastomas and polar spongioblastomas are too rare to predict their natural history. Some hypotheses are proposed concerning the evolution and interrelationship of malignant glial tumors of the cerebellum.     

Multiple Malignant Salivary Gland Neoplasms: Mucoepidermoid Carcinoma of Palate and Adenoid Cystic Carcinoma of Floor of Mouth

Salivary gland tumors usually occur as single lesions. To have more than one tumor is unusual. We report a case of an adult male who presented with a mucoepidermoid carcinoma involving the minor salivary glands of the palate at age 57 years, followed by an adenoid cystic carcinoma of the floor of mouth at age 63 years. The patient later succumbed to non-Hodgkin lymphoma at age 72 years. There are 31 acceptable cases of multiple malignant salivary gland neoplasms reported in the world literature. Multiple malignant tumors of the same histologic type are more common than those of different histologic type. Bilateral acinic cell adenocarcinoma was the most frequent combination of multiple salivary gland malignancy, accounting for 14 cases (10 synchronous and four metachronous). All involved the parotid glands bilaterally with the exception of one case that involved parotid and submandibular gland. Polymorphous low-grade adenocarcinoma accounted for three of the four cases of multiple malignant tumors involving minor salivary glands. Individuals with a history of malignancy are at risk for the development of additional malignant tumors and should receive appropriate clinical follow-up.

Conservative management of mesenchymal hamartomas of the chest wall

Mesenchymal hamartomas of the chest wall are rare benign lesions usually discovered in infancy. The authors present their experience with 3 cases. All of these cases were managed initially conservatively, although 1 child required a thoracotomy and partial tumour resection at 5 months of age because of respiratory compromise. The other 2 children have now reached 5 and 6 years of age with the tumors becoming less prominent. The authors believe many cases can be managed conservatively because malignant change has not been reported, and the lesions often become relatively smaller as the child grows.     

Clinical observations and the planning of therapy for sacrococcygeal tumors

Amongst sacrococcygeal tumors, teratoma is seen often while other histologic types are rare. It is apparent that the less obvious intrapelvic tumors can cause obstructive symptoms and are usually diagnosed late. Twenty cases of sacrococcygeal tumor, including 14 teratomas (9 benign, 5 malignant) and 6 others (3 benign, 3 malignant) were treated at Tokyo Medical College and Shizuoka Children's Hospital over the last 23 years. The majority of the benign tumors were readily excised, but one case of benign teratoma was difficult to excise and died of bleeding. The resectability of malignant tumors was low, but aggressive multiagent chemotherapy, radiotherapy and second look operation made it possible to increase the survival. One case is alive and has been disease free for 11 years. Recently combined therapy by chemotherapy and surgery has improved the survival rate although some problems still remain.     

Inflammatory pseudotumors in children

Inflammatory pseudotumors are so named because they mimic malignant tumors clinically and radiologically. Most often seen in the lungs of young adults, they consist of localized proliferations of mononuclear inflammatory cells and myofibroblasts. There are scattered reports of these tumors occurring in various sites in children. We report five cases of these rare lesions in children; four arising intraabdominally and one in the lung. In contrast to the usual presentation in adulthood, these children were all previously healthy. One child, with the tumor arising from the urinary bladder, was originally diagnosed as having a malignant sarcoma and underwent pelvic exenteration and chemotherapy for this subsequently-proven benign lesion. Local recurrence occurred in one case. Total excision is indicated and is usually possible without unacceptable morbidity. Our cases and a review of the literature point out the importance of pathologic differentiation of these lesions from malignancy with early appropriate surgery.     

Solitary eosinophilic granuloma of sternum

Primary bone tumors of the chest wall are uncommon, although a wide variety of both benign and malignant tumors arise within the chest wall. Among those tumors, sternal tumors are rare and usually malignant. We report an extremely rare case of eosinophilic granuloma developed in the sternum in a 30-year-old woman. She presented anterior chest pain and somewhat tender mass over the sternum. Chest roentgenogram, computed tomography scanning of the thorax, and total body bone scintigraphy revealed an isolated lytic lesion in the corpus sterni. A tru-cut biopsy of the mass exhibited the typical histologic appearance of eosinophilic granuloma. The solitary lesion was removed completely surgically. This rare condition should be kept in mind in differential diagnosis of sternal lesions.     

Clinical characteristics and survival for major salivary gland malignancies in children.

OBJECTIVE: Determine presentation and survival rates for malignant pediatric salivary gland neoplasms. METHODS: All cases of malignant neoplasms involving the parotid or submandibular gland in patients ages birth to 18 years were extracted from the Surveillance, Epidemiology, and End Results database (1988-2001). Variables included age, gender, tumor histology, size, follow-up time, and vital status. Kaplan-Meier survival curves were constructed. RESULTS: 113 primary salivary gland malignancies (103 parotid, 10 submandibular) were identified. Mean age at presentation was 13.2 years. Female:male ratio of 5:4. Mean tumor size was 2.5 cm. Among parotid tumors, there were 44 (43%) mucoepidermoid carcinomas and 35 (34%) acinic cell carcinomas. At a mean follow-up of 69.4 months, 6 (5.8%) patients with parotid malignancy were deceased; none of the submandibular malignancies were fatal. Mean Kaplan-Meier survival for parotid gland lesions was 153 months, with rhabdomyosarcomas exhibiting significantly worse survivals as compared to other malignancies (P < 0.001, log-rank test). CONCLUSIONS: Both epithelial and mesenchymal tumors present in the pediatric salivary gland. Survival for both parotid and submandibular gland malignancies is good in children. EBM rating: C-4.     

Pleural fibromas: a review based on 12 cases]

The authors report 12 cases of pleural fibroma, which is a rare benign tumor, usually discovered incidentally. However, these tumors are sometimes associated with a paraneoplastic syndrome, or may lead to compressive symptoms related to their large size. Radical resection is mandatory because recurrence may occur, sometimes several years later with a malignant behavior. Long-term follow-up is therefore necessary to detect any recurrence, particularly when certain prognostic factors are initially present.