Angiotropic large-cell lymphoma in a patient with adrenal insufficiency

Angiotropic large-cell lymphoma is a rare disorder characterized by a proliferation of malignant lymphoid cells within the lumina of small vessels. The skin and central nervous system are typically affected; however, involvement of other organs has been described. We document an unusual case of this disorder in a patient who suffered clinically significant adrenal insufficiency and subsequently died. Autopsy disclosed angiotropic large-cell lymphoma involving both adrenal glands.     

Angiotropic lymphoma with endocrine involvement

Aims : Two cases of angiotropic high-grade B-cell lymphoma are described in which involvement of endocrine glands was a prominent feature. Methods and results : One patient had marked unilateral adrenomegaly due to lymphoma within adrenal blood vessels. He is alive 5 years after presentation following combination chemotherapy. The diagnosis was made at autopsy in the second patient who presented with pyrexia of unknown origin and had hypothyroidism and a thyroid nodule. The thyroid gland and pituitary showed striking involvement by angiotropic lymphoma and the thyroid nodule was a cavernous haemangioma heavily colonized by lymphoma cells. Conclusions : Our cases illustrate endocrine organ manifestations of this rare disease. The condition may respond to combination chemotherapy.     

Angiotropic lymphoma with endocrine involvement

Aims: Two cases of angiotropic high-grade B-cell lymphoma are described in which involvement of endocrine glands was a prominent feature. Methods and results: One patient had marked unilateral adrenomegaly due to lymphoma within adrenal blood vessels. He is alive 5 years after presentation following combination chemotherapy. The diagnosis was made at autopsy in the second patient who presented with pyrexia of unknown origin and had hypothyroidism and a thyroid nodule. The thyroid gland and pituitary showed striking involvement by angiotropic lymphoma and the thyroid nodule was a cavernous haemangioma heavily colonized by lymphoma cells. Conclusions: Our cases illustrate endocrine organ manifestations of this rare disease. The condition may respond to combination chemotherapy.     

Ostensible oncocytoma of accessory lacrimal glands

Benign oncocytomas of the accessory lacrimal glands can be found in the lacrimal caruncle, plica semilunaris or conjunctival fornices, but are extremely rare. A series of 15 supposed oncocytomas of the ocular adnexa was reviewed retrospectively, and histological differences were noted with respect to the parotid gland counterpart. Lesions could be divided into three histological groups: (1) tumours composed of tubules lined by tall columnar epithelium with finely granular cytoplasm; the tubules often had dilated lumens containing mucinous secretion; (2) cystic tumours with prominent epithelial tufts projecting from much of the cyst wall; (3) tumours with solid areas composed of variably cuboidal or polygonal cells, largely in trabecular arrangement, and co-existing with the previously described tubular and cystic elements. A striking resemblance to Warthin's tumour without a lymphocytic component, such as may affect the parotid salivary gland, was noted in several tumours.     

Angiotropic lymphoma: an immunophenotypically and clinically heterogeneous lymphoma.

Angiotropic lymphoma (AL) is an uncommon lymphoma often presenting with nonspecific clinical features and having a high mortality rate. Although not specifically recognized by the Revised European-American Classification of Lymphoid Neoplasms, it likely will appear as a subtype of diffuse large B-cell lymphoma in the upcoming WHO classification. Some authors may also consider it to be a subtype of cutaneous lymphomas. Recent studies have reported an immunophenotypic heterogeneity of AL, and in rare instances, an association with other NHL. To further characterize AL, we studied the immunophenotype by immunohistochemistry for CD5, CD10, CD20, bcl-2, and bcl-6 in 18 cases of B-cell AL identified at three medical centers in North America. Bcl-2 gene rearrangement status by polymerase chain reaction and Epstein Barr virus status by in situ hybridization also were evaluated. Eight men and 10 women were identified with AL (median age 71 years). Eleven patients were diagnosed in life and seven were diagnosed at autopsy. Neurologic symptoms were the most common presentation, seen in six patients. Skin was the most commonly biopsied site. All showed classic intravascular localization; in two cases, there was also a minor diffuse large cell lymphoma component observed in some organs. Most (89%) of the cases expressed bcl-2 protein; CD10, bcl-6 and CD5 were each expressed in 22% of cases. Based on CD5 and CD10 expression, three major groups were evident: CD5-, CD10- (11 cases); CD5+, CD10- (3 cases), and CD5-, CD10+ (3 cases). Even though a follicle center lymphoma preceded the AL in one patient, we did not detect bcl-2 gene rearrangement in any of these cases. All cases were negative for Epstein Barr virus. Of the five treated with chemotherapy, two achieved a complete remission. Based on these findings, we conclude that ALs are clinically and immunophenotypically heterogeneous and may represent more than one pathogenetic entity. In some instances AL may be preceded by another lymphoproliferative disorder, raising the possibility that some cases of AL may represent a transformation from another type of lymphoma. Cutaneous manifestations of AL are common; however, it appears to be a systemic lymphoma. Although often fatal, patients with AL who are diagnosed early and treated with chemotherapy may achieve remission.     

Mucoepidermoid carcinoma of the lacrimal sac

Mucoepidermoid carcinoma of the lacrimal sac is a rare entity. We report a case of mucoepidermoid carcinoma arising in the lacrimal sac of a 30-year-old man who presented with an inner canthal mass. To our knowledge this is the first example of the cytopathology of this neoplasm in this anatomic site.     

Angiotropic lymphoma: report of a case with histiocytic features.

Angiotropic lymphoma, also known as intravascular lymphomatosis, is characterised by widespread intravascular proliferation of malignant lymphoid cells, usually without evidence of focal disease. A case of a 52 year old man referred for investigation of a two year history of pyrexia of unknown origin, skin rash and multiple organ failure is described. Angiotropic lymphoma was seen in gastric, colonic and skin biopsy specimens, and review of an earlier skin biopsy specimen showed similar morphological features. In contrast to previous cases which showed B or T cell differentiation, immunohistochemical examination was positive for histiocyte markers. Molecular studies showed no evidence of immunoglobulin heavy chain gene or T cell receptor gene rearrangement. The patient responded to combination chemotherapy, comprising cyclophosphamide, doxorubicin, etoposide, and prednisolone. This case highlights the fact that advanced lymphoma may be present without evidence of focal disease and that the diagnosis may be missed easily both clinically and histologically.     

Endoscopic dacryocystorhinostomy: creation of a large marsupialized lacrimal sac

This retrospective study describes and evaluates the effectiveness of a modified technique of conventional endoscopic dacryocystorhinostomy (DCR) that minimizes the obstruction of a neo-ostium by creating an enlarged marsupialized lacrimal sac using mucosal flaps. Forty-two patients who had undergone 46 endoscopic DCR at a tertiary medical center, from 2002 to 2004, for correction of lacrimal system obstruction were investigated. The surgical technique involves elevation of a nasal mucosal flap, full sac exposure using a power drill, and shaping of the mucosal flap to cover denuded bone and juxtapose exposed sac mucosa. Postoperative symptoms and endoscopic findings of the neo-ostium were evaluated. Mean duration of follow-up was 5.9 months. An eighty-three percent primary success rate was observed, without any serious complications. Obstruction of the neo-ostium with granulation tissue was observed in eight cases, among which six underwent revision with success in all cases. Overall, 44 (96%) of 46 cases experienced surgical successes. Endoscopic DCR, a procedure in which a large marsupialized lacrimal sac is created from mucosal flaps, yields a very satisfactory success rate with straightforward and highly successful revision available for those in whom the primary procedure yields a substandard result.     

Malignant angioendotheliomatosis (Angiotropic lymphoma) of the gallbladder

Summary We present a case of malignant angioendotheliomatosis of the gallbladder, the first reported. Diagnostic problems connected with this rare malignancy are underlined. Immunohistochemical studies were useful in providing further evidence of the lymphoid nature of the neoplasm and avoiding possible misdiagnosis. We suggest that the term angiotropic lymphoma might be more appropriate to define this malignancy.     

Angiotropic lymphoma (intravascular lymphomatosis)--2 case reports

Described are two cases of angiotropic lymphoma where eventually autopsy elucidated nonspecific neurologic symptoms. One patient suffering ambiguous encephalitic syndrome died three months later, the second one passed away after an unusually long three-year period of progressive dementia and cumulative motoric dysfunction. The autopsy disclosed pure intravascular malignant lymphoid aggregates (LCA, CD 20, Bcl 2-positive) in the brain and kidney of both patients. In the patients with the longer disease period, a dissemination to lung was also found. Definitive diagnosis was issued as a B cell type of angiotropic lymphoma. Skin, lymph nodes, spleen, and bone marrow were not affected in any case. The clinical differential diagnosis algorithm did not involve this rare etiology in these particular uncommon neurologic cases and even brain biopsy performed in both women did not recognize the substantiality of the disease.     

Angiotropic large-cell lymphoma presenting as pulmonary small vessel occlusive disease

Angiotropic lymphoma (AL) is an unusual variant of extranodal lymphoma, characterized by massive proliferation of neoplastic lymphoid cells almost exclusively within blood vessels. Whereas the lymphoid origin of this disease is widely accepted it still remains unclear whether AL is a distinct entity that originates in the blood vessels or whether it represents a form of secondary intravascular dissemination of a primary solid lymphoma. The present case is unusual because death by right heart failure owing to extensive intravascular proliferation of neoplastic cells and subsequent occlusion of pulmonary blood vessels has not been described so far. In addition, the patient had suffered from a solid deposit of a large-cell B-lymphoma months before the angiotropic manifestation, suggesting that AL might develop out of more common types of non-Hodgkin's lymphomas.     

An Epstein-Barr virus positive undifferentiated carcinoma in the lacrimal sac

We report a Chinese patient who presented with metastatic undifferentiated carcinoma in the cervical lymph node of unknown primary origin. Despite a raised IgA titre against Epstein-Barr virus capsid antigen, examination and biopsy of the nasopharynx were negative. Radiotherapy was given to the head and neck region with the orbit shielded. There was complete resolution of the metastatic lymph nodes. She developed proptosis of her left eye one year afterward when a large tumour was found in the lacrimal sac region. Review of the initial computerized tomography revealed a small soft tissue mass in the same region. The tumour was composed of undifferentiated carcinoma cells associated with dense lymphoid infiltrate. In situ hybridization for Epstein-Barr virus EBER RNA showed strong positive signals in the malignant cells. This is the first reported case of an Epstein-Barr virus positive undifferentiated carcinoma with lymphoid stroma in the lacrimal sac. In addition to the nasopharynx, salivary glands, nasal cavity and paranasal sinuses, the lacrimal sac should be considered as a potential primary site for Epstein-Barr virus positive metastatic undifferentiated carcinoma in the cervical lymph nodes.     

Angiotropic large cell lymphoma of the prostate gland: an immunohistochemical study

Malignant angioendotheliomatosis (MAE) is a rare disorder characterized by the intravascular proliferation of neoplastic mononuclear cells. Until recently, the cell of origin was uncertain; some investigators reported MAE to be lymphomatous in nature, while others claimed it to be of endothelial derivation. In the present unusual case, MAE was an incidental findings in the prostate of a patient with prostatic adenocarcinoma; it is shown to be a lymphoma of B-cell origin.     

血管内皮生长因子在慢性泪囊炎粘膜中的表达

目的:探讨血管内皮生长因子(VEGF)在泪囊慢性炎症组织中的表达,初步探讨慢性泪囊炎粘膜增生的发病机理。方法:应用免疫组化方法对12例慢性泪囊炎粘膜组织中VEGF的表达进行标记,分析粘膜不同部位VEGF的表达情况。选8例正常泪囊粘膜作为对照。结果:VEGF主要表达于上皮基底细胞层,阳性率为44.3%±7.6％,明显高于粘膜表皮和结缔组织间质(阳性率分别为16.9%±4.6％和15.2%±4.9％)(P<0.05),在正常粘膜中未见表达。结论:VEGF可能在慢性泪囊炎粘膜增生的病理过程中发挥重要的作用。