Skull base malignant lymphoma: a case report and review of the literature.

A case of primary malignant lymphoma of the skull base is reported. A 62-year-old man was admitted with headache and diplopia. Computed tomography and magnetic resonance imaging showed an abnormal mass in the right parasellar region. The final diagnosis after surgery was primary malignant lymphoma of the skull base. Primary malignant lymphomas sometimes occur in the central nervous system but those in the skull base are exceptional. The present paper presents just such a rare case of malignant lymphoma of the skull base. Diagnosis and combined therapy consisting of surgery, radiotherapy and chemotherapy for the disease are discussed, and the literature on recent clinical problems of central nervous system lymphomas is reviewed.     

Primary pulmonary Hodgkin's disease and the dilemma of E stage

Primary pulmonary Hodgkin's disease is rare. We report the case of a previously healthy 23-year-old woman who presented with isolated involvement of the right upper pulmonary lobe. Nodular sclerosing Hodgkin's disease was diagnosed after curative surgery. The clinical stage is felt to be IEBL+. The reasons for this staging, as opposed to a stage IV, are discussed. Adjuvant radiotherapy was given, delivering 39.6 grays in 22 courses of 1.8 grays. Three years after diagnosis, the patient is well and free of disease. A review of the literature indicates that the majority of primary pulmonary Hodgkin's disease present as a single mass amenable to curative surgery and radiation therapy. Chemotherapy can be reserved for the rare diffuse presentation.     

Primary oat cell carcinoma of the larynx: a case report and review of the literature.

A case of an extrapulmonary oat cell carcinoma arising in the larynx of a 45-year-old male is described. Ultrastructural study demonstrated numerous neurosecretory granules in the tumor cells identical to those described in oat cell carcinomas of the lung. Primary oat cell carcinomas of the larynx are highly malignant neoplasms. Of six previously reported acceptable cases of this rare entity, with adequate followup, all but one has died of tumor within 14 months of diagnosis. The present patient is alive without evidence of tumor 15 months following diagnosis. Aggressive therapy consisting of a combination of surgery, radiation therapy and systemic chemotherapy was utilized in this case. The use of such aggressive combination therapy appears indicated in cases of primary oat cell carcinoma of the larynx if improved survival is to be achieved.     

Angiosarcoma arising in an unirradiated breast with subsequent pituitary metastasis

Angiosarcoma of the breast is a rare condition with known risk factors. The Stewart-Treves syndrome describes angiosarcoma of the arm or breast in the setting of adjuvant radiation therapy (RT) after breast conservation surgery. Angiosarcoma of the breast, in the absence of RT, is rarer still. We present a unique case of angiosarcoma of the breast, diagnosed 6 years after conservative surgery for carcinoma of the breast when no adjuvant RT was given. Chronic postoperative lymphoedema in the breast delayed the investigation of skin changes, with an eventual diagnosis of angiosarcoma. An isolated pituitary metastasis 9 months from the diagnosis, the first such reported case, adds to the complexity of the case. The diagnosis and management of angiosarcoma of the breast is discussed. Angiosarcoma is a rare occurrence after breast conservative surgery. Postoperative lymphoedema can impede early diagnosis. The disease can display unique metastatic potential.     

Primary malignant melanoma arising in the parotid gland: case report and literature review

Melanoma of the head and neck is an extremely rare disease, characterized by difficult and late diagnosis and poor prognosis; moreover, the definition of primary melanomas of the salivary glands remains controversial. In this paper we describe an unusual case of primary melanoma arising in the parotid gland. A 60-year-old woman presented to us for the persistence of a parotid neoplasm diagnosed as carcinoma by fine-needle aspiration and treated with radiation therapy. Wide destructive surgery was performed and immunohistochemical analysis of the surgical specimen led to a diagnosis of melanoma. Complete screening excluded other primary tumor sites. After five years of follow-up the patient Is alive and free of disease. Here we report the diagnostic procedures leading to the diagnosis along with a critical analysis of the literature, emphasizing the difficulties in the diagnosis and classification of melanoma as a primary tumor in the parotid gland and the need for destructive surgery.     

Multimodality management of a locally advanced primary oesophageal melanoma with radical surgery and adjuvant chemoimmunoradiotherapy

We present the case of a 46-year-old woman diagnosed with a primary oesophageal melanoma (PEM), who was treated with radical surgery followed by combined chemoimmunotherapy (interferon, carboplatin, dacarbazine and external radiotherapy) and who achieved a complete response after this treatment. PEMs are rare malignancies, with less than 300 cases described in the literature. The main differential diagnosis is with metastases of skin or ocular malignant melanomas. They are usually diagnosed at advanced stages and prognosis is typically poor. The main treatment modality should be radical surgery. The role of adjuvant treatment is uncertain, although some long responses have been seen with the use of chemotherapy or immunotherapy alongside surgery.     

A case of ovarian carcinoma preceded by intracranial metastasis

A 28-year-old woman with primary ovarian carcinoma which was found with intracranial metastasis is reported. The patient was operated on because of metastatic brain tumor with unknown primary lesion. Subsequently, left ovarian tumor was found, and left salpingo-oophorectomy was performed. The histological diagnosis was endometrioid carcinoma. A review of the literature shows that intracranial metastasis by ovarian carcinoma is very rare. The incidence is lower than 2.1%. The present case in which intracranial metastasis manifested itself before primary ovarian carcinoma seems to be extremely rare.     

阑尾原发性腺癌晚期形成Krukenberg瘤的临床分析

目的:探讨阑尾原发性腺癌晚期形成Krukenberg瘤的临床特点、诊断及治疗。方法:分析1例表现为Krukenberg瘤的阑尾原发性腺癌患者的临床资料,并复习相关文献。结果:阑尾原发性腺癌晚期转移形成Krukenberg瘤的病例较为罕见,并且缺乏特异性临床表现,极易漏诊、误诊,术前或术中即明确诊断者极少见,绝大多数是通过术后组织病理学诊断而最终确诊。右半结肠切除术是阑尾原发性腺癌的首选治疗方式,术后辅以化疗等综合治疗。结论:以Krukenberg瘤为首发表现的病例在寻找原发癌灶时应考虑到原发性阑尾恶性肿瘤的可能。术中对阑尾的仔细探查是必要的,若发现阑尾形态异常改变除了及时行阑尾切除,术中还应对其进行冰冻病理检查以提高术中确诊率,指导手术治疗。     

Huge biatrial primary cardiac B-cell lymphoma resulting in bilateral atrioventricular valve obstruction

We report the case of a 48-year-old man with a clinical syndrome caused by both pulmonary and systemic venous congestion. Echocardiography indicated a huge tumor occupying both dilated atria almost completely, seeming to invade from the atrial septum. A test for HIV was negative. Our primary diagnosis was a biatrial myxoma. Urgent surgery was performed disclosing large cell B-cell primary cardiac lymphoma. The patient died 8 days later. Primary cardiac lymphoma is extremely rare in immunocompetent patients. Its rarity and heterogenous clinical presentation make diagnosis difficult. As tissue characterization is still beyond the capabilities of currently available echocardiography, a combination of examinations are indicated, when the clinical condition is relatively stable, in order to reach the exact diagnosis.     

原发性肝透明细胞癌1例临床分析及文献复习

目的：探讨原发性肝透明细胞癌的诊断和治疗方法。方法：分析我科收治的1例肝透明细胞癌治疗效果以及文献的相关报道。结果：1例54岁男性患者,表现为肝占位病变,经穿刺活检证实为肝透明细胞癌。经射频消融、经肝动脉介入治疗和γ刀治疗,患者生存27.0个月。结论：肝透明细胞癌的诊断须经病理免疫组化确诊,手术联合射频消融、经肝动脉介入化疗有助于提高肝透明细胞癌的治疗效果。     

原发性肝透明细胞癌1例临床分析及文献复习

目的:探讨原发性肝透明细胞癌的诊断和治疗方法.方法:分析我科收治的1例肝透明细胞癌治疗效果以及文献的相关报道.结果:1例54岁男性患者,表现为肝占位病变,经穿刺活检证实为肝透明细胞癌.经射频消融、经肝动脉介入治疗和γ刀治疗,患者生存27.0 个月.结论:肝透明细胞癌的诊断须经病理免疫组化确诊,手术联合射频消融、经肝动脉介入化疗有助于提高肝透明细胞癌的治疗效果.     

Primary ALK positive anaplastic large cell lymphoma of the pancreas

Here we present an unusual case of anaplastic lymphoma kinase positive anaplastic large cell lymphoma (ALCL), appearing as a primary tumor of the pancreas which invaded into the adjacent duodenal wall, causing upper gastrointestinal bleeding. After complete resection of the tumor (Whipple's operation), the patient received 4 cycles of CHOP chemotherapy. Currently, 2 years after diagnosis the patient still remains lymphoma free. Primary ALCL of the pancreas is very rare and has only been reported on one previous occasion. Nevertheless, lymphoma should be considered in the differential diagnosis of pancreatic tumors and an attempt to obtain tissue diagnosis is always necessary before continuing with radical surgery, especially in young patients.     

原发性卵巢非霍奇金淋巴瘤1例及相关文献复习

目的:探讨原发性卵巢弥漫性大B细胞淋巴瘤的病因、临床表现、诊断、治疗及预后。方法:报道1例原发性卵巢弥漫性大B细胞淋巴瘤的临床病理资料及术后免疫组化结果,并阅读及复习国内外相关文献。结果:该例患者为（右）卵巢弥漫性大B细胞淋巴瘤,积极治疗后现考虑肿瘤复发。结论:原发性卵巢弥漫性大B细胞淋巴瘤罕见,确诊依赖于术后病理及免疫组化,预后不佳,常采用以手术为主,辅以化疗和放疗的综合治疗。     

Fibromatosis of the Breast: A Case Report

Fibromatosis develops in many anatomic sites, but it rarely arises as a primary lesion in the breast. This lesion is locally invasive and frequently recurs after a local excision, but it has no potential for distant metastasis. In this report, we present a case of mammary fibromatosis which was closely similar to carcinoma in clinical, mammographic and ultrasonographic findings, thus leading us to breast conserving surgery. Despite being a rare disease, fibromatosis should be included in the differential diagnosis of younger patients (the age of the present case was 51, and the mean ages of patients with fibromatosis ranges from37 to 49) with abnormal changes on physical examinations and imaging studies.     

Klinefelter's syndrome and extragonadal germ cell tumors

A 25-year-old man presented with diffuse metastatic pure choriocarcinoma, thyrotoxicosis, and cardiac tamponade. No discernable testicular primary tumor was found. The patient's peripheral blood karyotype was 47, XXY and phenotypic features of Klinefelter's syndrome were present. The patient was treated with aggressive combination chemotherapy followed by salvage surgery and remains in complete remission 3 years after diagnosis. Pure choriocarcinoma, although rare as a primary testicular neoplasm, accounts for 15% of extragonadal germ cell tumors in general and 30% of germ cell tumors in patients with Klinefelter's syndrome. Historically, the diagnosis of pure choriocarcinoma has been thought to convey a very poor prognosis. The occurrence of hyperthyroidism is unique to tumors containing choriocarcinomatous elements and the management of this disorder is discussed. Treatment of extragonadal germ cell tumors is also discussed with special reference to the roles of combination chemotherapy and salvage surgery.     

PEG site metastasis in oropharyngeal squamous cell carcinoma managed with cetuximab and radiotherapy: A case report

Percutaneous endoscopic gastrostomy (PEG) tube insertion is commonplace in head and neck cancer (HNC) patients. A rare but serious complication of PEG insertion in HNC is PEG site metastasis (PSM), which may precipitate rapid deterioration. We present the first case of PSM in a HNC patient managed without chemoradiotherapy and/or surgery, but rather with concurrent radiotherapy and cetuximab, followed by second-line pembrolizumab. Following histopathologic diagnosis of PSM, positron emission tomography confirmed primary site recurrence and multiple metastases in the axilla, abdomen and pelvis, managed palliatively with focal abdominal wall radiotherapy, pembrolizumab and carboplatin. The patient deteriorated and passed away 20 months after initial HNC diagnosis, 5 months after confirmation of PSM. Patients and clinicians should be aware of PSM in HNC. Though a proven prevention strategy is yet to be confirmed, prompt PSM diagnosis spares the patient unnecessary antibiotics for presumed infection and suggests the possibility of intra-abdominal metastases.     

Primary intradural myxoid chondrosarcoma: a case report and review in the literature

Summary Objective and importance: Chondrosarcomas are extremely rare cartilaginous tumours that typically usually are associated with bone. Therapeutic experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. The exact origin of rare intradural spinal chondrosarcomas remains obscure. We report the first case in the literature of a primary intradural myxoid chondrosarcoma. Clinical presentation: This 40-year-old man experienced a 3 month history with back pain. The results of his neurological examination were normal. Magnetic resonance imaging (MRI)demonstrated at the T12 level intradural tumour. We could not identify this lesion as chondrosarcoma in preoperative period. Intervention: At surgery, a mass found attached solely to pia mater, with a normal arachnoid and dura mater overlying was seen. The mass was excised completely and microscopic examination identified a myxomatous chondrosarcoma. The postoperative course was unremercable. But a histological examination revealed primary myxoid chondrosarcoma. Experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. We especially discussed to the histological examination. Conclusion: The differential diagnosis considered in the present case included meningioma, plasmacytoma, and non-neoplastic intradural spinal cord lesion. We emphasize the benefit of surgical resection without radiotherapy and/or chemotherapy. This case presents the first case in the literature of an primary spinal intradural myxoid chondrosarcoma.     

A Primary Sacral Melanoma of Unknown Origin: A Case Report

Malignant melanoma caused by malignant transformation of melanocytes is associated with high mortality and is difficult to manage. Metastasis is not uncommon (up to 31% of all cases) and is closely associated with a poor prognosis. Although rare (4–5%), extracutaneous melanoma has been reported; however, primary malignant melanoma of the sacrum is extremely rare (only three case reports to date). Here, we present a 51-year-old patient who underwent surgical treatment for a lesion of the spinal canal and associated bony structures; extensive aggressive resection was required. She underwent partial sacrectomy and lumbo-iliac fixation (to maintain spinal stability). Pathology revealed malignant melanoma. We discuss the diagnosis, surgical intervention, and postoperative follow-up, which may assist clinicians. Although metastatic malignant melanoma is usually fatal, primary extracutaneous melanoma of the spine may respond well to surgery and adjuvant radiotherapy.     

Thyroid carcinoma showing thymus-like differentiation: Case presentation of a young man

Ectopic thymic tissue can be present in the thyroid gland and a carcinoma showing thymus-like differentiation (CASTLE) may arise from such tissue. We are reported the case of a 26-year-old man with CASTLE, with cervical subcutaneous nodules relapse, who showed a good response to treatment with surgery, chemotherapy and radiotherapy. The problematic aspect of this case was the diagnosis; only on review were we able to make a final diagnosis. CASTLE is a very rare neoplasm. It is important to differentiate this cancer from others tumors such as primary or metastatic squamous cell carcinoma of the head and neck or squamous cell thyroid carcinoma, because the therapy and prognosis are different. Diagnosis is complicated and requires careful histological analysis (CD5- and P63-positive with presence of Hassall’s corpuscles); unfortunately there is no gold standard treatment so, in this case, we administered a sandwich of chemotherapy and radiotherapy.