scholarly journals Retroperitoneal extramedullary hematopoietic pseudotumor in ataxia–telangiectasia

Rare Tumors ◽  
2018 ◽  
Vol 10 ◽  
pp. 203636131878972
Author(s):  
Sean James Judge ◽  
Trevor A Plescia ◽  
Cyrus P Bateni ◽  
Morgan A Darrow ◽  
Christopher P Evans ◽  
...  
Keyword(s):  
Natural History ◽  
Ataxia Telangiectasia ◽  
Patient Population ◽  
Hepatic Dysfunction ◽  
Malignant Tumors ◽  
Retroperitoneal Tumor ◽  
Retroperitoneal Sarcoma ◽  
Pathologic Evaluation ◽  
First Case ◽  
Cancer Types

Ataxia–telangiectasia confers a significant increase in the development of several cancer types, most commonly leukemia and lymphoma. However, as the natural history for these patients is evolving and their lifespan is increasing, there is the potential for the development of additional uncommon tumors in an already rare patient population. We report the first case, to our knowledge, of an incidental retroperitoneal tumor in a 26-year-old woman undergoing evaluation for hepatic dysfunction. The mass was suspicious for retroperitoneal sarcoma, but proved to be an extramedullary hematopoietic pseudotumor after extensive pathologic evaluation. The changing landscape of neoplasms associated with ataxia–telangiectasia is discussed with emphasis on previously underreported benign and malignant tumors.

scholarly journals Intraductal Carcinoma of the Parotid Gland Presenting as Parapharyngeal Mass

2020 ◽  
Vol 5 (3) ◽  
pp. 1-7
Author(s):  
Yamato Oki ◽  
Hiromitsu Hatakeyama ◽  
Masako Otani ◽  
Hidetaka Ikemiyagi ◽  
Masanori Komatsu ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Parotid Gland ◽  
Salivary Glands ◽  
Parapharyngeal Space ◽  
Malignant Tumors ◽  
Relevant Literature ◽  
Ct Scans ◽  
Intraductal Carcinoma ◽  
First Case

Intraductal carcinomas are rare, malignant tumors that arise from the salivary glands. They commonly grow from the parotid gland and no cases growing from the parapharyngeal space have been reported to date. We report a 76-year-old man who was inadvertently found to have a parapharyngeal lesion by CT scans and MR imaging. The tumor was resected through an upper neck approach and diagnosed histopathologically as intraductal carcinoma. As far as we are aware, this is the first case of intraductal carcinoma arising from the parapharyngeal space. Here, we describe the management of this disease together with a review of the relevant literature.

Primary stromal sarcoma of breast: A case report and literature review

2021 ◽  
pp. 1-7
Author(s):  
Mohammad Al-Wiswasy ◽  
Mahmoud Al-Balas ◽  
Raith Al-Saffar ◽  
Hamzeh Al-Balas
Keyword(s):  
Malignant Tumors ◽  
Histopathological Examination ◽  
Prognostic Significance ◽  
Total Duration ◽  
Young Patients ◽  
Soft Tissue Sarcomas ◽  
Breast Mass ◽  
Resection Margins ◽  
Stromal Sarcoma ◽  
First Case

INTRODUCTION: Primary breast sarcoma (PBS) was first described in 1887 as a rare heterogeneous neoplasm arising from the mesenchymal tissue of the mammary gland accounting for less than 5% of all body soft-tissue sarcomas and less than 0.1% of all malignant tumors of the breast. CASE PRESENTATION: A 31-year old lady presented with left breast mass which she felt four years before during which the mass increased in size from 1 to 6 cm in the largest diameter, diagnosed clinically as a benign fibroadenoma without any further cytological or histopathological confirmation. Histopathological examination of the excised breast mass reveals undifferentiated, primary stromal sarcoma of the breast (PSSB), which was followed by mastectomy three weeks later with reconstructive breast surgery with a total duration of follow-up of 3 years thereafter. This is the first case of PSSB reported in Jordan. DISCUSSION/CONCLUSION: PSSB is the generic term given to malignant breast tumors thought to arise from the specialized mesenchymal stroma of the breast but lacking an epithelial component with a phylloides pattern. PSSB is difficult to diagnose preoperatively due to its rarity and inadequate imaging methods to establish an exact diagnosis. The histology of the patient mass may be the leading factor for the management of these tumors. Even in very young patients, a progressively growing breast mass should alert the clinician to investigate for malignancy and verify the results by biopsy. Surgery with adequate resection margins represents the only potentially curative modality with prognostic significance. Adjuvant chemotherapy and radiotherapy are not very beneficial. The prognosis is dismal for patients with lymph node involvement and the size of the tumor has a lesser bearing on the outcome.

Penile Lymphoepithelioma-Like Carcinoma: A Rare Case With PD-L1 Expression

2021 ◽  
pp. 106689692098834
Author(s):  
Raquel Machado-Neves ◽  
Bernardo Teixeira ◽  
Elsa Fonseca ◽  
Pedro Valente ◽  
Joaquim Lindoro ◽  
...  
Keyword(s):  
Human Papillomavirus ◽  
Tumor Cells ◽  
Rare Case ◽  
Malignant Tumors ◽  
Squamous Cell Carcinomas ◽  
The Third ◽  
The Past ◽  
First Case ◽  
Lymphoplasmacytic Infiltrate

Most malignant tumors of the penis are squamous cell carcinomas (SCC), being divided in 2 groups, one human papillomavirus (HPV)-related and another non-HPV-related, with lymphoepithelioma-like carcinoma (LELC) being one of the rarest HPV-related SCC. In this article, we report a case of a 50-year-old man who presented testicular swelling and pain for the past 3 months. A penile mass was identified, and the patient was submitted to a total penectomy. The penectomy specimen showed an ulcerated lesion at the glans reaching the cavernous bodies. Microscopic examination showed undifferentiated epithelial cells with syncytial growth pattern mix with a dense lymphoplasmacytic infiltrate, consistent with LELC. The tumor cells expressed p16 and all 3 different clones of PDL1 (22C3, SP263, and SP142). The patient is alive and well with a follow-up of 3 months. To our knowledge, this is the third LELC of the penis reported in literature and the first case reported with PDL1 expression.

scholarly journals Synchronous intrathyroidal parathyroid carcinoma and thyroid carcinoma: case report and review of the literature

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nadia De Falco ◽  
Giuseppe Santangelo ◽  
Fabrizio Chirico ◽  
Angelo Cangiano ◽  
Maria Giulia Sommella ◽  
...  
Keyword(s):  
Literature Review ◽  
Thyroid Carcinoma ◽  
Papillary Carcinoma ◽  
Parathyroid Carcinoma ◽  
Malignant Tumors ◽  
Gamma Probe ◽  
Differentiated Thyroid Carcinoma ◽  
Thyroid Carcinomas ◽  
First Case ◽  
Well Differentiated

Abstract Background Parathyroid carcinoma is a rare endocrine malignancy, rarer when synchronous with a non medullary well differentiated thyroid carcinoma. Parathyroid carcinoma accounts of 0.005% of all malignant tumors and it is responsible for less than 1% of primary hyperparathyroidism. The intrathyroidal localization of a parathyroid gland is not frequent with a reported prevalence of 0.2%. Carcinoma of parathyroids with intrathyroidal localization represents an even rarer finding, reported in only 16 cases described in literature. The rare constellation of synchronous parathyroid and thyroid carcinomas has prompted us to report our experience and perform literature review. Case presentation We herein report a case of a 63-years-old man with multinodular goiter and biochemical diagnosis of hyperparathyroidism. Total thyroidectomy with radio-guide technique using gamma probe after intraoperative sesta-MIBI administration and intraoperative PTH level was performed. The high radiation levels in the posterior thyroid lobe discovered an intrathyroidal parathyroid. Microscopic examination revealed a parathyroid main cell carcinoma at the posterior thyroidal left basal lobe, a classic papillary carcinoma at the same lobe and follicular variant of papillary carcinoma at the thyroidal right lobe. To the best of our knowledge, this is the first case documenting a synchronous multicentric non medullary thyroid carcinomas and intrathyroidal parathyroid carcinoma. Conclusions Our experience was reported and literature review underlining challenging difficulties in diagnostic workup and surgical management was carried out.

scholarly journals Spinal Intradural, Extramedullary Ependymoma with Astrocytoma Component: A Case Report and Review of the Literature

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Gene M. Weinstein ◽  
Knarik Arkun ◽  
James Kryzanski ◽  
Michael Lanfranchi ◽  
Gaurav K. Gupta ◽  
...  
Keyword(s):  
Back Pain ◽  
Case Report ◽  
Lower Back Pain ◽  
Low Grade ◽  
Review Of The Literature ◽  
Pathologic Evaluation ◽  
Lower Back ◽  
Spinal Lesions ◽  
First Case ◽  
Intradural Extramedullary

Ependymomas are common spinal lesions, with the vast majority arising in an intramedullary location. Several cases have been described in the literature of ependymomas in an intradural, extramedullary location. The authors present a case of a 56-year-old female who presented with several weeks of lower back pain and weakness. MRI revealed an intradural, extramedullary enhancing mass at L1-L2. The mass was successfully resected surgically. Pathologic evaluation revealed a low grade glioma with components of both ependymoma and pilocytic astrocytoma with MUTYH G382D mutation. Extramedullary ependymomas are very rare tumors. To the authors’ knowledge, this is the first case of ependymoma/astrocytoma collision tumors described in an extramedullary location.

scholarly journals Angiomyolipoma with caval extension and regional nodal involvement: Aggressive behaviour or just rare natural history? Case report and review of literature

2014 ◽  
Vol 8 (3-4) ◽  
pp. 276 ◽  
Author(s):  
Kamaljot Singh Kaler ◽  
Rebekah Rittberg ◽  
Darrel Edmond Drachenberg
Keyword(s):  
Renal Cell Carcinoma ◽  
Case Report ◽  
Natural History ◽  
Renal Cell ◽  
Young Female ◽  
Renal Angiomyolipoma ◽  
Review Of Literature ◽  
Nodal Involvement ◽  
Regional Lymph Nodes ◽  
First Case

Renal angiomyolipoma (AML) is predominantly a non-aggressive benign tumour. Cases of more aggressive AMLs are present in the literature. We present 2 cases of aggressive AML behaviour. The first case is an AML with vascular extension in a young female and the second case is of AML found in regional lymph nodes in a female with a left renal AML and renal cell carcinoma.

scholarly journals Targeting Liver Cancer Stem Cells: An Alternative Therapeutic Approach for Liver Cancer

Cancers ◽  
2020 ◽  
Vol 12 (10) ◽  
pp. 2746
Author(s):  
Hwa-Yong Lee ◽  
In-Sun Hong
Keyword(s):  
Stem Cell ◽  
Liver Cancer ◽  
Signaling Pathways ◽  
Therapeutic Strategy ◽  
Malignant Tumors ◽  
Current Knowledge ◽  
Therapeutic Approaches ◽  
Effective Therapeutic Strategy ◽  
Cancer Types ◽  
The Brain

The first report of cancer stem cell (CSC) from Bruce et al. has demonstrated the relatively rare population of stem-like cells in acute myeloid leukemia (AML). The discovery of leukemic CSCs prompted further identification of CSCs in multiple types of solid tumor. Recently, extensive research has attempted to identity CSCs in multiple types of solid tumors in the brain, colon, head and neck, liver, and lung. Based on these studies, we hypothesize that the initiation and progression of most malignant tumors rely largely on the CSC population. Recent studies indicated that stem cell-related markers or signaling pathways, such as aldehyde dehydrogenase (ALDH), CD133, epithelial cell adhesion molecule (EpCAM), Wnt/β-catenin signaling, and Notch signaling, contribute to the initiation and progression of various liver cancer types. Importantly, CSCs are markedly resistant to conventional therapeutic approaches and current targeted therapeutics. Therefore, it is believed that selectively targeting specific markers and/or signaling pathways of hepatic CSCs is an effective therapeutic strategy for treating chemotherapy-resistant liver cancer. Here, we provide an overview of the current knowledge on the hepatic CSC hypothesis and discuss the specific surface markers and critical signaling pathways involved in the development and maintenance of hepatic CSC subpopulations.

Electron Microscopy in the Diagnosis of Liposarcoma and Fibrosarcoma of the Larynx

1979 ◽  
Vol 88 (6) ◽  
pp. 867-871 ◽  
Author(s):  
David N. Tobey ◽  
Roger F. Wheelis ◽  
C. Thomas Yarington
Keyword(s):  
Electron Microscopy ◽  
Light Microscopy ◽  
Accurate Determination ◽  
Final Diagnosis ◽  
Primary Lesion ◽  
Low Grade ◽  
Pathologic Evaluation ◽  
First Case ◽  
Per Se

Electron microscopy (EM) was useful in establishing the final diagnosis in specific instances of liposarcoma and fibrosarcoma of the larynx. In the first case light microscopy revealed cells that met the histologic criteria of a low grade liposarcoma; EM revealed cells that displayed a varied ultrastructural differentiation; thus the tumor was classified as a mixed mesenchymal tumor rather than liposarcoma per se. In the second case, pathologic evaluation indicated a grade 2 fibrosarcoma. EM revealed malignant mesenchymal cells that closely recapitulated normal fibroblasts (no epithelial differentiation was present). This kind of information enables more accurate determination of the source of the primary lesion than results of light microscopy alone.

scholarly journals Ureteroscopy-Assisted Biopsy for a Retroperitoneal Tumor: A Case Report

2015 ◽  
Vol 8 (2) ◽  
pp. 279-284
Author(s):  
Yoko Maeda ◽  
Takashi Kawahara ◽  
Mikiko Tanabe ◽  
Yohei Kumano ◽  
Mari Ohtaka ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Open Surgery ◽  
Retroperitoneal Fibrosis ◽  
Retroperitoneal Tumor ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Surgical Biopsy ◽  
Case Presentation ◽  
First Case ◽  
Open Surgical Biopsy ◽  
Enhanced Computed Tomography

Introduction: Retroperitoneal tumor is often seen in urology clinical practice. To diagnose the tumor, tumor specimens must be obtained. However, in some cases, the tumor is penetrated by vessels around the ureter, and it may be difficult to detect the optimal spot for obtaining a specimen, even when performing open surgery. Case Presentation: A 53-year-old male patient was referred to our hospital for further examination of left back pain due to hydronephrosis. Enhanced computed tomography demonstrated ureter stenosis in front of the ilium, which was surrounded by a retroperitoneal tumor. The tumor was penetrated by blood vessels; therefore, we performed an open surgical biopsy on the suspicion of a retroperitoneal tumor using ureteroscopic assistance. The diagnosis of idiopathic retroperitoneal fibrosis was made according to the biopsy. Conclusion: We herein report the first case of a ureteroscopy-assisted biopsy for the pathological diagnosis of a retroperitoneal tumor.

Monoclonal Gammopathy of the Immunoglobulin a Class in a Two-Year-Old Girl with Ataxia Telangiectasia

1998 ◽  
Vol 1 (4) ◽  
pp. 319-321 ◽  
Author(s):  
P.S. McDonald ◽  
D. Cora-Bramble ◽  
L. De Palma
Keyword(s):  
Ataxia Telangiectasia ◽  
Monoclonal Gammopathy ◽  
Unusual Case ◽  
Immunoglobulin A ◽  
Normal Serum ◽  
Neurologic Disorder ◽  
A Value ◽  
First Case ◽  
Immunofixation Electrophoresis ◽  
Restricted Mobility

Ataxia telangiectasia is an autosomal recessive neurologic disorder which is frequently associated with a deficiency of IgA immunoglobulin. We report an unusual case of monoclonal gammopathy of the IgA kappa type in a 2-year-old female patient newly diagnosed with ataxia telangiectasia. Quantitative analysis of the patient's immunoglobulins revealed a marked elevation in the IgA fraction with a value of 672 mg/dL (normal 14–123 mg/dL). The IgG and IgM fractions were normal. Serum protein electrophoresis showed a band of restricted mobility present in the gamma region, which was identified as a monoclonal IgA kappa immunoglobulin on immunofixation electrophoresis. This is the first case report of a patient with ataxia telangiectasia associated with an IgA monoclonal gammopathy.

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