Non-epithelial tumors of gastrointestinal tract: diagnosis and surgical treatment

Non-epithelial tumors of the gastrointestinal tract (GIT) are rare diseases, their rate is less than 2.6% of all tumors of this localization. Analysis of 271 clinical cases of non-epithelial tumors demonstrated that most of them (n = 174) belonged to smooth muscle tumors. Features of growth and localization of benign and malignant non-epithelial tumors were demonstrated. It was revealed that only morphologic study (frequently with histochemical methods) verified the diagnosis of neoplasm. The choice of the scope of surgical intervention depends on the localization, spread and morphology of tumor. Difficulties of verification before surgery lead to active surgical policy for non-epithelial GIT tumors.     

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??Endoscopic ultrasound in the diagnosis of pancreatic neuroendocrine tumors SHU Hui-jun, YANG Ai-ming.Department of Gastroenterology??Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730??China
Corresponding author: YANG Ai-ming,E-mail:yangaiming@medmail.com.cn
Abstract Pancreatic neuroendocrine tumors (PNETs) are rare tumors with malignant potential. Endoscopic ultrasound (EUS) has been shown to be of great importance in the preoperative localization and diagnosis of PNETs and may likely be superior to other imaging techniques in the assessment of small tumors. The usefulness of EUS as a primary exploration after US or CT has been posited for tumor diagnosis and localization before surgery.     

超声内镜在胰腺神经内分泌肿瘤诊治中的价值

胰腺神经内分泌肿瘤（PNETs）定位诊断的准确性是决定病人手术成败和预后的关键。文献报道,超声内镜（EUS）对PNETs的定位诊断具有很高的敏感性,已成为术前定位诊断的重要手段。     

Glioblastoma multiforme of the pineal region

A case of glioblastoma multiforme restricted to the pineal region is presented. Although glioblastomas of the pineal region are exceedingly rare, they have to be considered in the differential diagnosis of tumors at this localization.     

A rare etiology of rectal bleeding: Meckel's leiomyoblastoma. Value of celiomesenteric arteriography

A rare case of leiomyoblastoma developing in Meckel's diverticulum and revealed by massive rectorrhagia is reported. The characteristic clinicopathologic features of these tumors in their small intestine localization are described and the value of celiomesenteric arteriography emphasized for their topographic and even nosologic diagnosis.     

Primary liposarcoma of the fibular head: A rare location for a rare tumor: A case report

IntroductionPrimary bone liposarcoma are extremely rare tumors and their location in the fibular head is exceptional.Case reportA 19 year-old patient with a pain on the lateral of the proximal leg. The X-ray found an osteolytic lesion; MRI of the knee revealed a proximal epiphyseal-metaphyseal lesion process of the right fibula contours with cortical lysis and local medullary damage. The pathological study, follow by the immunohistochemical exam and the FISH concluded in an amplification of MDM2 (Murine double minute 2) confirming the presence of a liposarcoma.DiscussionThe primary bone localization of liposarcomas remains rare. Their localization at the level of the fibular head is exceptional. The proximal fibula is mainly affected by benign tumors, in particular giant cell. Malignant tumors localized to the head of the fibula as well as aggressive benign tumors most often require en bloc resection.ConclusionAlthough rare, primary liposarcomas can localize to the head of the fibula. It is necessary to establish a clinical, radiological and histological diagnosis for adequate management.     

Aneurysmal bone cysts in the cranial vault and base of skull

Three cases of aneurysmal bone cyst of the skull are reported. The localization in the base of the skull (two cases) is extremely rare and simulates a space-occupying intracerebral lesion. The clinical and microscopic findings, and their significance for the differential diagnosis from malignant brain tumors, are described.     

Malignant tumors of the gastrointestinal autonomic nervous system (GAN tumors or plexosarcomas). Report of a case

The plexosarcomas or gastrointestinal autonomic nerve tumors (GAN tumors) are very rare neoplasms originating from the gastrointestinal autonomic nervous system. They have been differentiated from the other gastrointestinal stromal tumors due to ultrastructural features showed by electronic microscopy. Their mesenteric localization rare than the gastric and intestinal localization, and it gives a very poor and specific clinical symptomatology. It's very difficult to make a correct diagnosis. The most important prognostic factors influencing the survival seem to be the tumor size and the mitotic activity. The authors describe a case of multiple Plexosarcomas occurred in a young patient one year after a surgical intervention for ileal resection to remove a benign schwannoma. This patient wasn't a carrier of multiple Neurofibromatosis. The diagnostic difficulties before surgical operation are stressed and the importance of surgery as the only diagnostic and therapeutic choice is underlined, even if local recurrences occurred in more than 50% of the treated cases.     

Ostéome ostéoïde de l’os naviculaire tarsien: à propos d’un cas et revue de la littérature

Osteoid osteoma is a benign tumour that usually affects the long bones. Its localization to the tarsal navicular bone of the foot is rare. We report a case of a 46-year-old man with an 18-month history of persistent foot pain whose diagnosis was difficult and established late because of a clinical presentation resembling arthritis. The patient underwent an en bloc excision of the nidus, reporting immediate pain relief and experiencing no recurrence of the lesion or symptoms 33 months after the procedure.     

Tumor adenomatoide paratesticular: una serie de nueve casos

IntroductionParatesticular tumors are rare. Most of them are benign, and adenomatoid tumors are most common.These tumors sometimes infiltrate the testicular parenchyma and require differential diagnosis with malignant tumors. In such cases, intraoperative biopsy allows for performing conservative surgery.Materials and methodsA retrospective study of nine patients with paratesticular adenomatoid tumors seen during a nine-year period (2000-2008) is reported.Results and conclusionsPatient age (mean, 49.6 years) and most common initial signs (tender nodule) are reported. The tumor most commonly occurred as a small, usually oval, nodule in the tail of epididymis.Our series included a case each of intraparenchymal tumor of the testis and tumor of the tunica vaginalis. Diagnosis was initially made based on a ultrasound scan and subsequently confirmed by histology. Differential diagnosis and surgical treatment, performed in all patients, are reported, and also the pathological features of surgical specimens     

Intra-abdominal desmoid tumor

Aggressive fibromatosis (desmoid tumor) are rare connective tissue tumors that occur sporadically or in association with familial adenomatous polyposis. The etiology is unknown and clinical findings depend on growth into neighboring structures. Biopsy is required to establish the diagnosis. The treatment of choice is surgery. We report a case with unusual localization in this form of presentation. The patient remains asymptomatic 15 months after surgery.     

Administration of bisphosphonate for hypercalcemia associated with oral cancer

Fanconi Anemia is a rare autosomal recessive disorder characterized by various congenital malformations, progressive bone marrow failure at a very young age and of solid tumors development. The authors present a rare case of a squamous cell carcinoma of the hard palate in a Fanconi Anaemia patient. The atypical clinical manifestation rendered the diagnosis more difficult. This case, for age of appearance, sex and localization, is unique in international literature. We recommend a quarterly follow up of the oral-rhino-pharynx complex in FA patients and to consider as carcinomas, all oral lesions that last more than two weeks.     

Gastrointestinal stromal tumor with primary hepatic unique location--clinical case

The gastrointestinal stromal tumors are mesenchymal tumors whose primary extradigestive location is very rare (less than 10% primary liver localization). We present a clinical case of primary hepatic location of GIST in a 28 year-old patient. The discovery of this tumor is a chance, the patient presenting for non-specific dyspeptic syndrome and epigastralgia. During the presentation an abdominal ultrasound is performed which identifies an whell-delineated hepatic mass - 5/4 cm. Clinical and paraclinical investigations (CT, EDS, EDI, examination of the intestinal lumen with the videocapsula), confirm the diagnosis of unique hepatic mass of segments III-IV. The diagnosis is confirmed intraoperatory and we perform an atypical liver resection of segments III-IV (with 1 cm safety-margin). The histopatologic exam: GIST.     

Tumoren des Bewegungsapparats

Musculoskeletal tumors, particularly bone neoplasms, are very rare. Diagnosis and treatment require an interdisciplinary concept as well as wide experience of all physicians involved. The final histopathologic diagnosis should not be confirmed without information regarding the patient’s age, exact localization, and radiological findings. The requirements of additional diagnostic procedures (molecular pathology) have to be taken into consideration when planning a biopsy.     

Kyste dermoïde intraorbitaire : un cas

Dermoid cysts account for 3–4 % of primary orbital tumors. The intraorbital location is relatively rare. The authors report a case of a 12-year-old male with an intraorbital dermoid cyst revealed by progressive right proptosis. The imaging aspects (CT scan and MRI) were consistent with intraconic dermoid cyst. The tumor was extirpated via a subfrontal approach. Histopathologic examination confirmed the diagnosis of dermoid cyst. The authors discuss the clinical symptoms, radiological aspects, and management.     

Tumeur neuroectodermique primitive des parties molles de l’index chez l’adulte. À propos d’un cas

The primitive neuroectodermal tumours (PNET) of soft tissues belong to the Ewing's tumors family and affects particularly the child. The localization of the disease at the extremities is very rare within the adult population and raises the problem of differential diagnosis with others tumors of the soft tissues. We report the case of a 48-year-old patient with a localized tumor, at the level of the second right finger, of six months evolution. The biopsy showed the infiltrating nature of the tumour; and the diagnosis of (PNET) was confirmed after the histological and immunohistochemical study. The extension assessment was negative and the patient had an amputation of the second and third rays of the right hand. Four years afterwards, the patient showed no recurrence or metastases.     

Malignant mesenchymoma of the pleura

Malignant mesenchymomas are rare soft tissue tumors of mesenchymal origin. We report a case of pleural localization with liposarcomatous, leiomyosarcomatous and osteocartilaginous elements. The treatment associated surgical resection, chemotherapy and radiotherapy. Sixteen months after the diagnosis a metastatic retroperitoneal sarcoma with osteosarcomatous differentiation appeared without any sign of thoracic recurrence. A surgical resection was performed, but a rapid retroperitoneal recurrence led to death.     

Incidence and demographics of giant cell tumor of bone in The Netherlands: First nationwide Pathology Registry Study

Background and purpose — Giant cell tumors of bone (GCT-B) are rare, locally aggressive tumors characterized by an abundance of giant cells. Incidence studies for GCT-B are rare. This is the first study using a fully automated 100% covering pathology database, the nationwide Dutch Pathology Registry (17 million inhabitants), PALGA, to calculate incidence rates for GCT-B.

Patients and methods — From PALGA, all pathology excerpts were retrieved for patients diagnosed with GCT-B, giant cell tumors of tenosynovium, and giant cell tumors of soft tissue between January 1, 2009 and December 31, 2013. The incidence of GCT-B was calculated.

Results — In total, 8,156 excerpts of 5,922 patients were retrieved; these included 138 first GCT-B diagnosis. For GCT-B the incidence was 1.7 per million inhabitants per year with a male to female ratio of 1:1.38 and a median age of 35 years (9–77). Most common localization was the femur (35%), followed by the tibia (18%). No differences in localization according to age and sex were found. The incidence rate of local recurrence was 0.40 per million inhabitants per year.

Interpretation — This is the first nationwide study reporting the incidence of GCT-B, based on a nationwide pathology database with 100% coverage of pathology departments. Current incidence calculations are based only on doctor-driven registries. We confirmed that GCT-B is a rare disease with an incidence that is slightly higher than previously published. The relatively young median age of patients and the high incidence of recurrence stresses the importance of developing more effective treatments for this disease.     

A case of incidentally detected Castleman's disease with retroperitoneal paravertebral localization.

Castleman's disease, or angiofollicular lymph node hyperplasia, is a fairly rare benign tumor of lymphoid origin. The expected localization is mediastinum and rarely retroperitoneum. An asymptomatic case is reported with multimodality imaging and postoperative findings. The lesion was detected incidentally on routine chest radiogram. Surgical exploration revealed a retroperitoneal mass and the mass was resected successfully through a thoracoabdominal flank incision. Castleman's disease should be included in the list of differential diagnosis of retroperitoneal masses which are mostly malignant tumors.     

Intramedullary schwannoma. A case report

The intramedullary localization of schwannoma is rare, corresponding to 0.3% of all intraspinal tumors. We report a case of intramedullary schwannoma without symptoms suggestive of neurofibromatosis. This patient presented with symptoms of spinal compression. Total removal of the tumor was achieved. The literature is reviewed about of this rare localization of schwannoma.