scholarly journals Papillary cystadenoma of right testis: Case report and literature review

2017 ◽  
Vol 4 (2) ◽  
pp. 8
Author(s):  
Otobo O. Fidelis ◽  
Ikpi Edet ◽  
Enakirerhi Glen ◽  
Isiwele M. Edoise ◽  
Omotosho Ayodele ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Case Report ◽  
Malignant Tumors ◽  
Benign Neoplasm ◽  
Adenomatoid Tumor ◽  
Von Hippel Lindau ◽  
Male Undergraduate ◽  
History Of ◽  
Papillary Cystadenoma ◽  
Vhl Disease

Testicular cystadenoma is ranked the second commonest benign neoplasm. Other benign epididymal neoplasms include adenomatoid tumor (most common), leiomyoma, serous (nonpapillary) cystadenoma, cavernous hemangioma, and melanotic neuroectodermal tumor. Adenocarcinoma, mesothelioma, and metastatic renal cell carcinoma are malignant tumors that can affect the epididymis. A 24-year-old male undergraduate with a 3-month history of mildly tender right testicular swelling histologically diagnosed as papillary cystadenoma is presented. This case is presented from our locality as the first of its’ kind; and because it can be a possible manifestation of other diseases like von Hippel- Lindau (VHL) disease.

scholarly journals Papillary Cystadenoma: An Incidental Finding in Tubal Ligation

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Tabitha Lynn Ward ◽  
Neda Zarrin-Khameh
Keyword(s):  
Autosomal Dominant ◽  
Malignant Tumors ◽  
Incidental Finding ◽  
Close Association ◽  
Tubal Ligation ◽  
Adenomatoid Tumor ◽  
Autosomal Dominant Disorder ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Papillary Cystadenoma

von Hippel-Lindau disease (vHLD) is a rare autosomal dominant disorder with multiple benign and malignant tumors of different organs. We report a papillary cystadenoma of the mesosalpinx found in close association with an adenomatoid tumor discovered incidentally following tubal ligation in a patient with vHLD.

scholarly journals Sorafenib treatement for recurrent stage T1 bilateral renal cell carcinoma in patients with Von Hippel- Lindau disease: A case report and literature review

2015 ◽  
Vol 9 (9-10) ◽  
pp. 651 ◽  
Author(s):  
Kyung Hwa Choi ◽  
Young Dong Yu ◽  
Moon Hyung Kang ◽  
Dong Soo Park
Keyword(s):  
Renal Cell Carcinoma ◽  
Case Report ◽  
Cell Carcinoma ◽  
Surgical Resection ◽  
Renal Cell ◽  
Sorafenib Treatment ◽  
Von Hippel Lindau ◽  
First Case ◽  
Vhl Disease ◽  
Stage T1

Renal cell carcinoma (RCC) with Von Hippel-Lindau (VHL) syndrome is associated with multiple recurrences and a young age at diagnosis. Therefore the primary goal of treatment is to stabilize the disease, minimizing the surgical resection and preserving the renal function in the patients with VHL who have developing RCC nodules after initial treatment. This is the first case report of VHL disease, with long-term stable disease, treated with a half dose of sorafenib after surgical resection and radiofrequency ablation for multiple recurrent stage T1 masses. We discuss the efficacy and safety of low-dose sorafenib treatment and review RCC in a patient with VHL disease.

Papillary Cystadenoma of the Epididymis

2010 ◽  
Vol 134 (4) ◽  
pp. 630-633 ◽  
Author(s):  
Karen J. Odrzywolski ◽  
Sanjay Mukhopadhyay
Keyword(s):  
Renal Cell Carcinoma ◽  
Hypoxia Inducible Factor ◽  
Benign Neoplasm ◽  
Surgical Excision ◽  
Precursor Lesions ◽  
Adult Males ◽  
Von Hippel Lindau ◽  
Immunohistochemical Markers ◽  
Von Hippel Lindau Disease ◽  
Papillary Cystadenoma

Abstract Papillary cystadenoma is a rare benign neoplasm of the epididymis, occurring mainly in young adult males. More than one-third of the cases reported in the literature have occurred in patients with von Hippel-Lindau disease. Conversely, epididymal nodules presumed to be papillary cystadenomas are found in one-third of males with von Hippel-Lindau disease. The association is stronger for bilateral tumors. The pathogenesis involves loss of the von Hippel-Lindau gene resulting in overexpression of the angiogenic protein “hypoxia-inducible factor.” Papillary cystadenoma is of mesonephric derivation. It originates in the efferent ductules of the head of the epididymis in the form of tiny precursor lesions. Histologically, papillary cystadenoma is characterized by cystic spaces with intracystic papillary projections lined by clear cells, with a resultant resemblance to renal cell carcinoma. Immunohistochemical markers may facilitate the distinction between the 2 tumors. Treatment consists of surgical excision and the prognosis is excellent.

scholarly journals Hereditary renal cell carcinoma associated with von Hippel–Lindau disease: a description of a Nova Scotia cohort

2013 ◽  
Vol 3 (1) ◽  
pp. 32 ◽  
Author(s):  
Shannon Bradley ◽  
Nadine Dumas ◽  
Mark Ludman ◽  
Lori Wood
Keyword(s):  
Renal Cell Carcinoma ◽  
Nova Scotia ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Prise En Charge ◽  
Mortality Data ◽  
Malignant Tumours ◽  
Multiple Sources ◽  
Von Hippel Lindau ◽  
Vhl Disease

Background: von Hippel–Lindau (VHL) disease is an autosomaldominant condition characterized by the development of benignand malignant tumours, including cases of renal cell carcinoma(RCC). Early detection of RCC through routine surveillance canlead to decreased morbidity and mortality. Data on the numberof patients in Nova Scotia (NS) who have VHL disease, diseasemanifestations and the frequency and mode of the surveillancehave not previously been collected or reported. This project wasdesigned to obtain that information.Methods: The number and management of patients with VHL diseasewas determined by multiple sources: the Maritime MedicalGenetics Service, patient charts, and pathology, radiology and laboratorydata. The actual surveillance being performed was comparedwith that recommended in the literature.Results: Twenty-one patients from 11 families in NS were identified.Manifestations included cases of RCC (31.6%), central nervoussystem (CNS) hemangioblastoma (73.7%), retinal hemangioma(47.4%), renal cyst (47.4%) and pheochromocytoma (10.5%).Of the 6 patients with RCC, 4 had bilateral tumours, 2 requiredkidney transplants and 1 developed metastatic disease. Routinesurveillance was being done for the CNS in 62.5% of patients,retina in 47.4%, abdomen in 43.8% and urine catecholaminesin only 10.5%. Only 1 of the 6 patients who developed RCCwas undergoing routine abdominal imaging. Surveillance investigationswere ordered by a number of different specialists.Conclusion: Patients with VHL disease in NS have a number of manifestationsassociated with their disease, including RCC, in a similarfrequency to that reported in the literature. The surveillanceof these patients is suboptimal in frequency and coordination.von Hippel–Lindau disease is a complex condition that requiresa coordinated approach to care to ensure proper surveillance andtreatment. Our study highlights current deficiencies and offersan enormous opportunity for improvement.Généralités : La maladie de von Hippel-Lindau (VHL) est une maladieà transmission autosomique dominante caractérisée par la formationde tumeurs bénignes et malignes, dont l’hypernéphrome.Le dépistage précoce de l’hypernéphrome par des examens régulierspeut amener une réduction de la morbidité et de la mortalité. Onne sait pas combien de personnes sont atteintes de VHL enNouvelle-Écosse, quelles sont les manifestations de la maladie chezces patients et quels tests de dépistage sont effectués et à quellefréquence. Le projet décrit ici visait à obtenir ces renseignements.Méthodologie : Le nombre et la méthode de prise en charge despatients atteints de VHL ont été établis à l’aide de plusieurs sources :la Clinique de génétique médicale des Maritimes, des dossiers depatients, des rapports de pathologie et de radiologie et des analysesde laboratoire. Les méthodes de surveillance mises en placeont été comparées aux méthodes recommandées dans la littératuremédicale.Résultats : Vingt et un patients de 11 familles de Nouvelle-Écosseont été cernés. Les manifestations incluaient : hypernéphrome(31,6 %), hémangioblastomes siégeant au niveau du SNC (73,7 %),hémangiomes rétiniens (47,4 %), kystes rénaux (47,4 %) etphéochromocytomes (10,5 %). Sur les six patients porteurs d’unhypernéphrome, 4 avaient des tumeurs bilatérales, 2 ont eu besoind’une transplantation rénale et un patient a présenté des métastases.De tous les patients atteints de VHL, 62,5 % ont subi destests réguliers de dépistage au niveau du SNC, 47,4 %, au niveaude la rétine, 43,8 %, au niveau de l’abdomen, et seulement 10,5 %des patients ont subi des tests réguliers de dépistage des catécholaminesurinaires. Sur les 6 cas d’hypernéphrome, un seulementsubissait des épreuves régulières d’imagerie au niveau del’abdomen. Les tests de dépistage avaient été prescrits par différentsspécialistes.Conclusion : Les cas de VHL en Nouvelle-Écosse présentent un certainnombre de manifestations liées à cette maladie, dont l’hypernéphrome,à une fréquence proche de celle mentionnée dansla littérature. La fréquence et la coordination des épreuves dedépistage sont sous-optimales. La maladie de VHL est une affectioncomplexe nécessitant une bonne coordination des soinsafin d’assurer une surveillance et un traitement adéquats. Cetteétude montre les lacunes actuelles et pointe vers des améliorationssubstantielles.

scholarly journals Giant retroperitoneal lipoma: a case report

2003 ◽  
Vol 40 (4) ◽  
pp. 251-255 ◽  
Author(s):  
Carlos Augusto Real Martinez ◽  
Rogério Tadeu Palma ◽  
Jaques Waisberg
Keyword(s):  
Case Report ◽  
Histological Study ◽  
Abdominal Mass ◽  
Benign Neoplasm ◽  
Large Mass ◽  
White Female ◽  
Ascending Colon ◽  
Abdominal Ultrasonography ◽  
History Of ◽  
History Of Pain

BACKGROUND: Retroperitoneal lipoma is an extremely rare neoplasm. AIMS: The authors report a case of giant retroperitoneal lipoma in a 32-year-old white female, with a history of pain and an abdominal mass over a 2-year period. Total abdominal ultrasonography and barium enema showed a large mass located in the retroperitoneal space behind the ascending colon. Laparotomy showed a large encapsulated tumor measuring 20 x 13 x 10 cm and weighing 3.400 g. The histological study revealed a benign neoplasm of fatty cells. CONCLUSION: The patient remains well 17 years after surgery, without recurrentce of the disease.

scholarly journals Olfactory Neuroblastoma: A Case Report and Review of the Literature

2005 ◽  
Vol 84 (3) ◽  
pp. 150-152 ◽  
Author(s):  
Shehzad Ghaffar ◽  
Iftikhar Salahuddin
Keyword(s):  
Case Report ◽  
Facial Pain ◽  
Elderly Woman ◽  
Malignant Tumors ◽  
Olfactory Neuroblastoma ◽  
Lateral Rhinotomy ◽  
Review Of The Literature ◽  
Postoperative Radiation ◽  
History Of

Malignant tumors of the nasal cavity are rare. We report the case of an elderly woman who consulted us with a 4-year history of progressive nasal obstruction, occasional epistaxis, facial pain, and watering of the eyes. A diagnosis of olfactory neuroblastoma was established by histopathology and confirmed by immunohistochemistry. On staging, the mass was classified as a Kadish stage B tumor. The mass was excised via a lateral rhinotomy approach, and the tumor was peeled away completely from the cribriform plate with endoscopes. The patient underwent postoperative radiation, and she was free of recurrence at follow-up 15 months later.

scholarly journals von Hippel-Lindau Disease: an Update

2019 ◽  
Vol 7 (4) ◽  
pp. 227-235 ◽  
Author(s):  
Eamonn R Maher ◽  
Richard N Sandford
Keyword(s):  
Natural History ◽  
Patient Outcomes ◽  
Molecular Characterisation ◽  
Von Hippel Lindau ◽  
Functional Characterisation ◽  
History Of ◽  
Vhl Protein ◽  
Von Hippel Lindau Disease ◽  
Vhl Disease ◽  
Improve Patient

Abstract Purpose of Review In this review, we discuss the key molecular and clinical developments in VHL disease that have the potential to impact on the natural history of the disease and improve patient outcomes. Recent Findings Identifiable mutations in VHL underlie most cases of VHL and define clear genotype-phenotype correlations. Detailed clinical and molecular characterisation has allowed the implementation of lifelong screening programmes that have improved clinical outcomes. Functional characterisation of the VHL protein complex has revealed its role in oxygen sensing and the mechanisms of tumourigenesis that are now being exploited to develop novel therapies for VHL and renal cancer. Summary The molecular and cellular landscape of VHL-associated tumours is revealing new opportunities to modify the natural history of the disease and develop therapies. Drugs are now entering clinical trials and combined with improved clinical and molecular diagnosis, and lifelong surveillance programmes, further progress towards reducing the morbidity and mortality associated with VHL disease is anticipated.

The natural history of hemangioblastomas of the central nervous system in patients with von Hippel—Lindau disease

2003 ◽  
Vol 98 (1) ◽  
pp. 82-94 ◽  
Author(s):  
John E. Wanebo ◽  
Russell R. Lonser ◽  
Gladys M. Glenn ◽  
Edward H. Oldfield
Keyword(s):  
Spinal Cord ◽  
Natural History ◽  
Mass Effect ◽  
Content Type ◽  
Von Hippel Lindau ◽  
History Of ◽  
The Central Nervous System ◽  
Brainstem Tumors ◽  
Vhl Disease ◽  
Fine Print

Object. The goals of this study were to define the natural history and growth pattern of hemangioblastomas of the central nervous system (CNS) that are associated with von Hippel—Lindau (VHL) disease and to correlate features of hemangioblastomas that are associated with the development of symptoms and the need for treatment. Methods. The authors reviewed serial magnetic resonance images and clinical histories of 160 consecutive patients with VHL disease who harbored CNS hemangioblastomas and serially measured the volumes of tumors and associated cysts. Six hundred fifty-five hemangioblastomas were identified in the cerebellum (250 tumors), brainstem (64 tumors, all of which were located in the posterior medulla oblongata), spinal cord (331 tumors, 96% of which were located in the posterior half of spinal cord), and the supratentorial brain (10 tumors). The symptoms were related to a mass effect. A serial increase in hemangioblastoma size was observed in cerebellar, brainstem, and spinal cord tumors as patients progressed from being asymptomatic to symptomatic and requiring surgery (p < 0.0001). Twenty-one (72%) of 29 symptom-producing cerebellar tumors had an associated cyst, whereas only 28 (13%) of 221 nonsymptomatic cerebellar tumors had tumor-associated cysts (p < 0.0001). Nine (75%) of 12 symptomatic brainstem tumors had associated cysts, compared with only four (8%) of 52 nonsymptomatic brainstem lesions (p < 0.0001). By the time the symptoms appeared and surgery was required, the cyst was larger than the causative tumor; cerebellar and brainstem cysts measured 34 and 19 times the size of their associated tumors at surgery, respectively. Ninety-five percent of symptom-producing spinal hemangioblastomas were associated with syringomyelia. The clinical circumstance was dynamic. Among the 88 patients who had undergone serial imaging for 6 months or longer (median 32 months), 164 (44%) of 373 hemangioblastomas and 37 (67%) of 55 tumor-associated cysts enlarged. No tumors or cysts spontaneously diminished in size. Symptomatic cerebellar and brainstem tumors grew at rates six and nine times greater, respectively, than asymptomatic tumors in the same regions. Cysts enlarged seven (cerebellum) and 15 (brainstem) times faster than the hemangioblastomas causing them. Hemangioblastomas frequently demonstrated a pattern of growth in which they would enlarge for a period of time (growth phase) and then stabilize in a period of arrested growth (quiescent phase). Of 69 patients with documented tumor growth, 18 (26%) harbored tumors with at least two growth phases. Of 160 patients with hemangioblastomas, 34 patients (median follow up 51 months) were found to have 115 new hemangioblastomas and 15 patients new tumor-associated cysts. Conclusions. In this study the authors define the natural history of CNS hemangioblastomas associated with VHL disease. Not only were cysts commonly associated with cerebellar, brainstem, and spinal hemangioblastomas, the pace of enlargement was much faster for cysts than for hemangioblastomas. By the time symptoms appeared, the majority of mass effect—producing symptoms derived from the cyst, rather than from the tumor causing the cyst. These tumors often have multiple periods of tumor growth separated by periods of arrested growth, and many untreated tumors may remain the same size for several years. These characteristics must be considered when determining the optimal timing of screening for individual patients and for evaluating the timing and results of treatment.

scholarly journals Adenomatoid Tumor of the Adrenal Gland: Report of Two Cases and Review of the Literature

2021 ◽  
Vol 12 ◽  
Author(s):  
Jiexia Guan ◽  
Chang Zhao ◽  
Hengming Li ◽  
Wenjing Zhang ◽  
Weizhen Lin ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Tumor Cells ◽  
Malignant Tumors ◽  
English Literature ◽  
Benign Neoplasm ◽  
Adenomatoid Tumor ◽  
Ki 67 ◽  
Prognostic Features ◽  
Signet Ring ◽  
Male Patients

Adenomatoid tumor (AT) is an uncommon benign neoplasm of mesothelial origin, usually occurring in the female and male genital tracts. Extragenital localization such as the adrenal gland is extremely rare. Until now, only 39 cases of adrenal AT have been reported in the English literature. Here we report two novel cases of adrenal AT that occurred in male patients aged 30 and 31 years. The tumors were discovered incidentally by computed tomography (CT). Macroscopically, the tumors were unilateral and solid, and the greatest dimension of the tumors was 3.5 and 8.0 cm, respectively. Histologically, the tumors consisted of angiomatoid, cystic, and solid patterns and infiltrated the adrenal cortical or medullary tissue. The tumor cells had low nuclear/cytoplasmic ratio, with no pathological mitosis or nuclear pleomorphism. Thread-like bridging strands and signet-ring-like cells could be seen. Immunohistochemically, the tumor cells were positive for epithelial markers (AE1/AE3, CK7) and mesothelial markers (D2-40, calretinin, and WT-1). The Ki-67 index was approximately 1 and 2%, respectively. The differential diagnosis of adrenal AT includes a variety of benign and malignant tumors. The patients had neither local recurrence nor distant metastasis at 21 and 8 months after removal of the tumor. In the literature review, we comprehensively summarized the clinical, morphological, immunohistochemical, and prognostic features of adrenal AT. Adrenal ATs are morphologically and immunophenotypically identical to those that occur in the genital tracts. Combining the histology with immunohistochemical profiles is very supportive in reaching the diagnosis of this benign tumor, helping to avoid misdiagnosis and overtreatment.

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