Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer

2021 ◽  
Vol 12 (8) ◽  
Author(s):  
Jennifer L. Spicer, MA, PA-C
Keyword(s):  
Abdominal Mass ◽  
Treatment Strategies ◽  
Distant Metastases ◽  
Radical Resection ◽  
Primary Treatment ◽  
Low Grade ◽  
Appreciable Change ◽  
Presenting Symptoms ◽  
Treatment Information ◽  
The Right

Retroperitoneal liposarcomas (RLPS) are rare tumors that have variable clinical behavior and complex treatment strategies based on presentation, histopathology, and genomics. Early identification is critical, and complete surgical resection remains the primary treatment, although chemotherapy and radiation are used on individual bases. Presenting symptoms are often nonspecific; therefore, a high degree of suspicion is essential for early diagnosis. In this report, the management of a 37-year-old otherwise healthy male with a large RLPS causing right groin/testicular pain is presented. After three evaluations in the emergency department, the patient was diagnosed and received two cycles of doxorubicin/ifosfamide/mesna (AIM) neoadjuvant chemotherapy. His physical exam on presentation for second opinion demonstrated a large palpable abdominal mass and fullness around the right spermatic cord. There was no appreciable change in tumor size or distant metastases on repeat scanning. Given some obstructive symptoms, a multidisciplinary team advised neoadjuvant radiation followed by radical resection of RLPS. Final pathology demonstrated a 31-cm grade II well-differentiated (WD) liposarcoma with low-grade dedifferentiation. Scattered foci of microscopic positive WD margins were noted, and the remainder of margins were negative. Genomic evaluation showed amplification of CDK4, MDM2, and FRS2. A concise literature review of common presentations, histopathology, genomics, and treatment information is discussed herein. Thorough physical exams, attention to subtle findings, appropriate medical imaging studies, and a high index of suspicion when evaluating vague symptomatology can lead to earlier diagnosis and treatment of RLPS, and ultimately better patient outcomes.

scholarly journals Nasopharyngeal Hyalinizing Clear Cell Carcinoma: A Case Report and Review of the Literature

2019 ◽  
Vol 10 ◽  
pp. 215265671988903 ◽  
Author(s):  
Madison J. Malfitano ◽  
Meghan N. Norris ◽  
Wesley H. Stepp ◽  
Griffin D. Santarelli ◽  
T. Danielle Samulski ◽  
...  
Keyword(s):  
Case Report ◽  
Salivary Glands ◽  
Clear Cell ◽  
Malignant Tumors ◽  
Treatment Strategies ◽  
Low Grade ◽  
Minor Salivary Glands ◽  
Presenting Symptoms ◽  
Nasopharyngeal Mass ◽  
The Right

Background Hyalinizing clear cell carcinomas (HCCCs) are rare, low-grade, malignant tumors which most often arise from the minor salivary glands primarily in palate and tongue but can arise in any location with minor salivary glands including the nasopharynx. Methods A case report of primary nasopharyngeal HCCC is presented. Because of the rarity of this tumor and location, a literature search was conducted to determine the most common presenting symptoms, treatment strategies, and outcomes. Results A 48-year-old man underwent biopsy of a 4.5 cm mass of the right nasopharynx with pathology suggesting an intermediate grade mucoepidermoid carcinoma. After discussing management with the patient, an endoscopic resection was performed. Final pathology revealed an HCCC which was confirmed after negative Mastermind-like 2 (MAML2) and positive Ewing sarcoma breakpoint region 1 (ESWR1) gene rearrangements on fluorescence in situ hybridization (FISH) studies. Literature review of other nasopharyngeal HCCC cases shows diverse presentation and overall excellent prognosis through surgical and radiation therapy. Conclusion HCCCs are rare, low-grade malignant tumors of the minor salivary glands and can present as a nasopharyngeal mass. Presenting symptoms are diverse but frequently involve otologic and sinonasal disturbances. HCCC is an indolent tumor with an excellent prognostic outcome when treated appropriately with surgical resection and adjuvant radiotherapy.

scholarly journals Mucoepidermoid Carcinoma of the Lung: A Case Report and Literature Review

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
S. Alsidawi ◽  
J. C. Morris ◽  
K. A. Wikenheiser-Brokamp ◽  
S. L. Starnes ◽  
N. A. Karim
Keyword(s):  
Lung Cancer ◽  
Surgical Resection ◽  
Mucoepidermoid Carcinoma ◽  
Primary Treatment ◽  
Pathological Examination ◽  
Low Grade ◽  
High Grade ◽  
Lobe Bronchus ◽  
Rare Type ◽  
The Right

Introduction. Mucoepidermoid carcinoma (MEC) of the lung is a rare form of lung cancer that is classified into low grade and high grade based on histological features. Surgical resection is the primary treatment for low-grade MEC with excellent outcomes, while high-grade MEC is a more aggressive form of malignancy.Clinical Case. We report a case of a 46-year-old woman who presented with dyspnea on exertion. Imaging studies revealed a mass involving the right upper lobe bronchus. Bronchoscopy, surgical resection, and pathological examination revealed a low-grade MEC with tumor-free margins. No adjuvant treatment was given.Discussion. Primary pulmonary MEC is a rare type of lung cancer with only few reported cases. This patient illustrates a typical presentation for low-grade MEC wherein surgical resection is considered curative. In contrast, high-grade MEC is a more aggressive malignancy with a poorer outcome. The role of targeted therapy directed against EGFR or a novel CRTC1-MAML2 fusion protein expressed in some high-grade tumors is yet to be determined.

scholarly journals Primary Adenoid Cystic Carcinoma of the Skin with Multiple Local Recurrences

2015 ◽  
Vol 8 (2) ◽  
pp. 251-255 ◽  
Author(s):  
Ivan Dimitrov Tonev ◽  
Yana Stoeva Pirgova ◽  
Nikolay Vladimirov Conev
Keyword(s):  
Adenoid Cystic Carcinoma ◽  
English Literature ◽  
Distant Metastases ◽  
Primary Treatment ◽  
Rare Tumor ◽  
Local Recurrences ◽  
Adenoid Cystic ◽  
Lymph Node Enlargement ◽  
The Right ◽  
Multiple Recurrences

Primary adenoid cystic carcinoma (PACC) of the skin is a rare tumor with fewer than 70 cases studied in detail in the English literature. This type of tumor shows a prolonged course and a growth pattern usually manifested by multiple local recurrences and has a low potential for distant metastases. The most important modality for primary treatment is surgical resection followed by radiotherapy. We report a woman aged 43 years at the time of diagnosis, who presented with a slow-growing nodule in the right axilla without lymph node enlargement. A wide local excision was performed, and the histology revealed an adenoid cystic carcinoma. During the next 24 years, another four local recurrences were excised (the last one in 2015) and confirmed histologically to be adenoid cystic carcinoma. The patient was given 44 Gy of radiotherapy after the second surgery in 1996. PACC of the skin is a rare tumor with insufficient data concerning the efficacy of the surgical technique and chemotherapy and radiotherapy treatment, even more so in the case of multiple recurrences. After the last recurrence, the patient was offered an active follow-up based on the long tumor-free intervals in the past and because the site of the primary tumor allowed further surgical excisions in future recurrences.

Epithelial Myoepithelial Carcinoma of the Nasal Cavity: Clinical, Histopathological, and Immunohistochemical Distinction of a Case Report

2017 ◽  
Vol 26 (4) ◽  
pp. 342-346 ◽  
Author(s):  
Sally Nguyen ◽  
Marjorie Perron ◽  
Sylvie Nadeau ◽  
Alexandre Nakao Odashiro ◽  
Marie-Noëlle Corriveau
Keyword(s):  
Nasal Cavity ◽  
Distant Metastases ◽  
Inferior Turbinate ◽  
Disease Recurrence ◽  
Myoepithelial Cells ◽  
Sinus Surgery ◽  
Low Grade ◽  
Oncocytic Variant ◽  
Epithelial Myoepithelial Carcinoma ◽  
The Right

Background. Epithelial myoepithelial carcinomas (EMCs) are rare low-grade salivary gland tumors. Here, we report the case of a 75-year-old man presenting with an oncocytic variant of EMC of the nasal cavity, initially diagnosed as an oncocytoma. Methods. Our patient underwent functional sinus surgery in 2012. On pathology, an oncocytic neoplasm was found in the right nasal cavity, characterized by fragments of uniform bland oncocytic cells with bilayered arrangement of nuclei. Immunohistochemical stains demonstrated biphasic cells: luminal epithelial and basal cell–type myoepithelial cells. The tumor was best diagnosed as an oncocytoma. In 2015, the patient presented with a recurrent right inferior turbinate lesion, compatible with oncocytic EMC. Results. The patient underwent oncological surgery and received adjuvant radiotherapy. He had no disease recurrence. Conclusion. Different variants of EMCs exist, such as oncocytic EMC. EMCs should be treated aggressively because they can be locally invasive, recur, and give rise to distant metastases.

scholarly journals P040 An unusual case of giant cell arteritis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Jessica Ellis ◽  
Keziah Austin ◽  
Sarah Emerson
Keyword(s):  
Inflammatory Markers ◽  
Illicit Drugs ◽  
Unusual Case ◽  
Temporal Arteritis ◽  
White Cell Count ◽  
Healthcare Providers ◽  
Temporal Artery ◽  
Low Grade ◽  
Temporal Artery Biopsy ◽  
The Right

Abstract Background/Aims  A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods  The case is discussed below. Results  Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion  GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure  J. Ellis: None. K. Austin: None. S. Emerson: None.

scholarly journals A Case of Atypical Bartonellosis in a 4-Year-Old Immunocompetent Child

2021 ◽  
Vol 9 (5) ◽  
pp. 950
Author(s):  
Chiara Sodini ◽  
Elena Mariotti Zani ◽  
Francesco Pecora ◽  
Cristiano Conte ◽  
Viviana Dora Patianna ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Bartonella Henselae ◽  
Delayed Diagnosis ◽  
Mitral Valve Regurgitation ◽  
Low Grade ◽  
Systemic Involvement ◽  
Mild Disease ◽  
Precise Diagnosis ◽  
Major Illness ◽  
The Right

In most cases, infection due to Bartonella henselae causes a mild disease presenting with a regional lymphadenopathy frequently associated with a low-grade fever, headache, poor appetite and exhaustion that spontaneously resolves itself in a few weeks. As the infection is generally transmitted by cats through scratching or biting, the disease is named cat scratch disease (CSD). However, in 5–20% of cases, mainly in immunocompromised patients, systemic involvement can occur and CSD may result in major illness. This report describes a case of systemic CSD diagnosed in an immunocompetent 4-year-old child that can be used as an example of the problems that pediatricians must solve to reach a diagnosis of atypical CSD. Despite the child’s lack of history suggesting any contact with cats and the absence of regional lymphadenopathy, the presence of a high fever, deterioration of their general condition, increased inflammatory biomarkers, hepatosplenic lesions (i.e., multiple abscesses), pericardial effusion with mild mitral valve regurgitation and a mild dilatation of the proximal and medial portion of the right coronary artery, seroconversion for B. henselae (IgG 1:256) supported the diagnosis of atypical CSD. Administration of oral azithromycin was initiated (10 mg/kg/die for 3 days) with a progressive normalization of clinical, laboratory and US hepatosplenic and cardiac findings. This case shows that the diagnosis of atypical CSD is challenging. The nonspecific, composite and variable clinical features of this disease require a careful evaluation in order to achieve a precise diagnosis and to avoid both a delayed diagnosis and therapy with a risk of negative evolution.

scholarly journals Provocation and prediction of visual peripersonal neglect-like symptoms in preoperative planning and during awake brain surgery

2021 ◽  
Author(s):  
Viktória Tamás ◽  
Gabriella Sebestyén ◽  
Szilvia Anett Nagy ◽  
Péter Zsolt Horváth ◽  
Ákos Mérei ◽  
...  
Keyword(s):  
Preoperative Planning ◽  
Right Hemisphere ◽  
Superior Temporal Gyrus ◽  
Low Grade ◽  
Navigated Transcranial Magnetic Stimulation ◽  
Resonance Imaging ◽  
Parietal Area ◽  
Routine Magnetic Resonance Imaging ◽  
Visuospatial Functions ◽  
The Right

AbstractNeglect is a severe neuropsychological/neurological deficit that usually develops due to lesions of the posterior inferior parietal area of the right hemisphere and is characterized by a lack of attention to the left side. Our case is a proven right-handed, 30-year-old female patient with a low-grade glioma, which was located in the temporo-opercular region and also in the superior temporal gyrus of the right hemisphere. Upon presurgical planning, the motor, language, and visuospatial functions were mapped. In order to achieve this, the protocol for routine magnetic resonance imaging and navigated transcranial magnetic stimulation has been expanded, accordingly.

Management of Recurrent or Progressing Skull Base Chondrosarcomas: Predictors of Long-Term Outcomes

2020 ◽  
Author(s):  
Jonathan D. Breshears ◽  
Franco DeMonte ◽  
Ahmed Habib ◽  
Paul W. Gidley ◽  
Shaan M. Raza
Keyword(s):  
Natural History ◽  
Skull Base ◽  
Radiation Treatment ◽  
Systemic Disease ◽  
Primary Treatment ◽  
Salvage Treatment ◽  
Low Grade ◽  
Related Factors ◽  
Factors Associated ◽  
Initial Recurrence

Abstract Background Skull base chondrosarcomas (CSA) are difficult tumors to cure and there is little data regarding salvage therapy. Objective This study aims to identify presentation and treatment-related factors which impact the progression free survival (PFS) and disease specific survival (DSS) for recurrent CSA, and to identify salvage treatment factors associated with successful restoration to the natural history following primary treatment. Methods This single-institution retrospective review included patients with recurrent/progressive CSA over a 25-year period. Survival analysis for factors impacting PFS and DSS was performed. Salvage treatment factors associated with achieving PFS ≥newly diagnosed median PFS were identified using univariate statistics. Analysis was performed on first recurrences and all recurrences combined. Results A total of 47 recurrence/progression events were analyzed from 17 patients (median two events/patient, range = 1–8). The overall PFS and DSS for the initial recurrence was 32 (range = 3–267) and 79 (range = 3–285) months, respectively. Conventional grade III or mesenchymal histology significantly predicted shorter PFS and DSS (p < 0.0001). After stratification by histology, previous radiation predicted shorter PFS for low-grade tumors (p = 0.009). Gross total resection (GTR) after a first time recurrence was significantly associated with successful salvage treatment (p < 0.05); however, this was rare. Conclusion In this series, high grade histology and prior radiation treatment negatively impacted salvage treatment outcomes, while GTR was associated with restoration to natural history following primary treatment. Careful consideration of histology, systemic disease status, previous treatments, and the anatomic extent of the skull base disease can optimize the outcomes of salvage intervention.

scholarly journals Spontaneous Resolution of Late-Onset, Symptomatic Fluid Collection Localized in the Meningioma Resection Cavity: A Case Report and Suggestion of Possible Pathogenesis

2021 ◽  
Vol 11 (3) ◽  
pp. 299
Author(s):  
Yeong Jin Kim ◽  
Tae-Young Jung ◽  
In-Young Kim ◽  
Shin Jung ◽  
Kyung-Sub Moon
Keyword(s):  
Brain Imaging ◽  
Late Onset ◽  
Fluid Collection ◽  
Good Condition ◽  
Treatment Strategies ◽  
Imaging Studies ◽  
Resection Cavity ◽  
Perilesional Edema ◽  
Posterior Neck ◽  
The Right

Postoperative complications after brain tumor surgery occur occasionally and it is important for clinicians to know how to properly manage each complication. Here, we described a rare case of late-onset, subdural fluid collection localized at the resection cavity that caused motor weakness after convexity meningioma resection, requiring differentiation from an abscess, to help clinicians determine treatment strategies. A 58-year-old right-handed female was admitted to the hospital with a headache and posterior neck pain. Brain computed tomography (CT) scans and magnetic resonance (MR) images showed a homogeneously enhanced, calcified, and multi-lobulated mass adjacent to the right motor strip without perilesional edema. The patient underwent surgery without incident or residual deficit and was discharged from the hospital in good condition. Six weeks after surgery, the patient complained of left arm monoparesis without infection-related symptoms. Brain imaging studies showed a localized fluid collection in the resection cavity with an enhanced margin and perilesional edema. Diffusion restriction was not detected. After three months of conservative treatment without surgery or antibiotics, she recovered from the neurologic deficits, and brain imaging studies showed the spontaneous regression of the fluid collection and perilesional edema. Late-onset, localized fluid collection at the resection cavity, which is similar to an abscess, could occur three to eight weeks after meningioma resection. When there are predisposing factors, including blood components and hemostatic materials in the surgical cavity, it is important for clinicians to understand this type of complication and choose conservative management as a feasible strategy.

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