Mesenchymal tumours and other neoplasms.

Author(s):  
F. Cian ◽  
P. Monti
Author(s):  
R.L. Martuza ◽  
T. Liszczak ◽  
A. Okun ◽  
T-Y Wang

Neurofibromatosis (NF) is an autosomal dominant genetic disorder with a prevalence of 1/3,000 births. The NF mutation causes multiple abnormalities of various cells of neural crest origin. Schwann cell tumors (neurofibromas, acoustic neuromas) are the most common feature of neurofibromatosis although meningiomas, gliomas, and other neoplasms may be seen. The schwann cell tumors commonly develop from the schwann cells associated with sensory or sympathetic nerves or their ganglia. Schwann cell tumors on ventral spinal roots or motor cranial nerves are much less common. Since the sensory neuron membrane is known to contain a mitogenic factor for schwann cells, we have postulated that neurofibromatosis may be due to an abnormal interaction between the nerve and the schwann cell and that this interaction may be hormonally modulated. To test this possibility a system has been developed in which an enriched schwannoma cell culture can be obtained and co-cultured with pure neurons.


Author(s):  
Andrzej Cieszanowski ◽  
Agnieszka Anysz-Grodzicka ◽  
Joanna Podgorska ◽  
Beata Jagielska ◽  
Jakub Pałucki

<P>Background: Primary Hepatic Epithelioid Haemangioendothelioma (HEHE) and Primary Hepatic Angiosarcoma (PHA) are rare mesenchymal tumours with different malignant potential. Whereas HEHE demonstrates low to intermediate malignant potential, PHA is an aggressive malignancy with poor prognosis. The knowledge of typical imaging features of these lesions may facilitate correct diagnosis; however, the ultimate diagnosis of HEHE and PHA is based on histopathologic examination. </P><P> Discussion: The most typical findings helpful in diagnosing HEHE are: Presence of multiple, confluent nodules located at the liver periphery (in young to middle-aged woman), retraction of the liver capsule, marked hyperintensity on T2-weighted images, “target-sign” appearance, progressive centripetal contrast enhancement, and relatively high Apparent Diffusion Coefficient (ADC) values. More than &#8805;50% of nodules are hyper- or isointense on Hepatobiliary Phase (HBP) images. Conclusion: The imaging features suggestive of PHA are: Occurrence of metastases (lungs, spleen) at the time of diagnosis, presence of a large dominant mass with smaller satellites, heterogeneity and areas of haemorrhage in a dominant mass, progressive contrast enhancement, slightly elevated ADC values as compared to other malignant liver tumours.</P>


1978 ◽  
Vol 87 (5_suppl) ◽  
pp. 5-10 ◽  
Author(s):  
Seymour R. Cohen ◽  
Stuart Siegel ◽  
Eva Heuser ◽  
Benjamin H. Landing ◽  
Susan Shen ◽  
...  

Lymphomatoid granulomatosis, a tumor-like process of unknown etiology, produced progressively destructive disease of the larynx, trachea and bronchi in an eight-year-old girl with acute lymphoblastic leukemia of five years duration. The leukemia had been in remission for 4½ years at the clinical onset of the lymphomatoid granulomatosis. Whether this occurrence suggests that lymphomatoid granulomatosis is a type of neoplasm, or is associated with immunologic depression, cannot be stated. Fortunately rare, and of poor prognosis, the disorder may become more frequent with improved survival rate of patients with leukemia and other neoplasms.


2019 ◽  
Vol 25 (1) ◽  
Author(s):  
Danielle Whiting ◽  
Ian Rudd ◽  
Amit Goel ◽  
Seshadri Sriprasad ◽  
Sanjeev Madaan

Abstract Background Angiomyolipomas are rare mesenchymal tumours arising from the perivascular epithelioid cells consisting of variable amounts of adipose, thick-walled blood vessels and smooth muscle cells. These benign tumours commonly occur in the kidney with only a few case reports of adrenal angiomyolipomas which have the potential to reach a large size and haemorrhage. Case presentation A 45-year-old lady presented with a 3-week history of right loin pain, nausea and vomiting. A CT scan revealed a right adrenal angiomyolipoma measuring 6.3 × 6.8 cm with associated haemorrhage. The lesion was successfully treated with right open adrenalectomy, and histology confirmed the diagnosis of adrenal angiomyolipoma. The patient remained well with no evidence of recurrence at the 36-month follow-up. Conclusion Adrenal angiomyolipomas are rare benign tumours that have the ability to reach a large size and potential to bleed. Here, we report the second case of spontaneous haemorrhage in an adrenal angiomyolipoma, which was successfully treated with open adrenalectomy.


2021 ◽  
Vol 9 ◽  
pp. 2050313X2110008
Author(s):  
Solange De Noon ◽  
Benjamin Ayres ◽  
Uday Patel ◽  
Rami Issa ◽  
Colan Maxwell Ho-Yen

Perivascular epithelioid cell neoplasms represent a group of uncommon mesenchymal tumours with as yet undiscovered benign counterpart. Although perivascular epithelioid cell neoplasms have been described arising in most organ systems as well as in soft tissue and bone, only a small number of perivascular epithelioid cell neoplasms have been reported in the bladder. To date, there is no agreed system for predicting the behaviour of these tumours. We describe a case of a perivascular epithelioid cell neoplasm of the bladder arising in a 57-year-old male and initially diagnosed on biopsy and present a review of the literature focussing on the pathological differential diagnosis and the importance of key histological features in conjunction with a broad immunohistochemical panel. This case report highlights the key features of bladder perivascular epithelioid cell neoplasms that distinguishes these rare neoplasms from other bladder lesions.


2012 ◽  
Vol 62 (2) ◽  
pp. 229-236 ◽  
Author(s):  
Maike Büttner ◽  
Verena Kufer ◽  
Kathrin Brunner ◽  
Arndt Hartmann ◽  
Kerstin Amann ◽  
...  

2013 ◽  
Vol 82 (4) ◽  
pp. 387-392 ◽  
Author(s):  
Lucia Frgelecová ◽  
Miša Škorič ◽  
Petr Fictum ◽  
Roman Husník

The aim of our study was to investigate histopathologically the incidence of gastrointestinal tract tumours in dogs in the Czech Republic in relation to their age, sex and breed. In total, 29 gastric and 45 intestinal tumours from 3827 gastrointestinal tract biopsies were investigated. The average age of dogs with gastric lymphoma, adenocarcinoma, and other mesenchymal tumours was 7.3 years, 9.5 years, and > 10 years, respectively. Breed predisposition to adenocarcinoma was evidenced in the Belgian Shepherd and Leonberger, to lymphoma in the Doberman (P < 0.01). The most frequent site of adenocarcinoma was antrum pyloricum (66.7%); lymphomas involved gastric wall diffusely. The average age of dogs with intestinal adenoma, adenocarcinoma, lymphoma, leiomyoma or leiomyosarcoma and carcinoid was 7.5, 8.5, 8.5, 12, and 14 years, respectively. The age predisposition to intestinal adenoma and lymphoma was 7–8 years (P < 0.01) and to adenocarcinoma 7–8 years (P < 0.05); mesenchymal tumours occurred at the age of 11–12 years (P < 0.01). Breed predisposition to intestinal adenoma was evidenced in the Pug, Leonberger and English Setter (P < 0.01), to adenocarcinoma in the English Setter (P < 0.01) and Hovawart (P < 0.05), to lymphoma in the Doberman and Hovawart (P < 0.05). The most frequent site of occurrence of intestinal tumours was the colorectal region. The study revealing breed, sex and age predisposition to gastrointestinal tumours in dogs has not been described yet in the literature.


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