An Unusual Cause of Proptosis: Orbital Metastasis of Paratesticular Leiomyosarcoma

Paratesticular leiomyosarcomas are uncommon malignant tumors that originate from smooth muscle. Paratesticular leiomyosarcoma shows aggressive tumor behavior due to a high incidence of local recurrence and distant metastasis. The orbit metastasis is also rare and associated with a poor prognosis. Clinical suspicion plays an essential role in the diagnosis of orbital leiomyosarcoma due to uncommon presentation. Comprehensive neuroimaging and histopathological work-up warranted for definitive diagnosis and management. To date, surgical resection remains the best curative treatment. However, leiomyosarcomas are usually friable and unencapsulated, which makes surgery challenging. Also, whether adjunctive chemotherapy will alter the long-term prognosis remains to be determined. Herein, we report the case of a young male who presented with proptosis and decreased vision, subsequently diagnosed with metastatic orbital leiomyosarcoma from a primary paratesticular tumor. We performed orbital enucleation with negative margins. The patient is currently under systemic, systemic pazopanib chemotherapy.

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What Every Primary Physician Should Know About the Postoperative Cardiac Patient

PEDIATRICS ◽

10.1542/peds.63.2.320 ◽

1979 ◽

Vol 63 (2) ◽

pp. 320-330

Author(s):

Arthur J. Moss

Keyword(s):

Cardiac Surgery ◽

Ductus Arteriosus ◽

Adult Life ◽

Primary Physician ◽

The Family ◽

High Incidence ◽

Local Physician ◽

Long Term Prognosis ◽

Parental Protectiveness

Many patients with the defects discussed here are under continuing postoperative observation by the cardiologist, and that is as it should be. This is particularly likely to be the case with tetralogy of Fallot and transposition of the great arteries. Some patients, however, are returned from distant cardiac centers, and the services of another cardiologist may not be readily available. Even if available, the cardiologist depends on the patient's local physician to detect early signs of trouble. Also, the family generally has a warmer relationship with the primary physician and looks to him for guidance and counsel. This assumes particular importance when one considers the high incidence of psychologic problems that tend to occur in children who have been subjected to cardiac surgery.63 These psychologic disturbances are attributed to such things as inability to compete physically, hospitalizations, family anxiety, and parental protectiveness or pampering. Most patients who have experienced cardiac surgery should be reevaluated periodically, preferably by an experienced cardiologist. Of all the lesions discussed here, patent ductus arteriosus has the best long-term prognosis. With this lesion, once the electrocardiogram and roentgenogram have returned to normal, it probably is no longer necessary to reevaluate the patient from a cardiac standpoint unless there is a change in the murmur or a new one appears. With all the other lesions, continued cardiac reevaluation is recommended, the exact interval to be determined by the age of the patient, the postoperative course, and the time elapsed from the date of operation. During periods of stress, such as pregnancy and later adult life, closer observation may be necessary.

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Adenosquamous Carcinoma of the Lateral Oropharyngeal Wall

Ear Nose & Throat Journal ◽

10.1177/014556131008901106 ◽

2010 ◽

Vol 89 (11) ◽

pp. E18-E21 ◽

Cited By ~ 2

Author(s):

Daniel P. Nadeau ◽

Paul C. Shick ◽

Robin Lindsay

Keyword(s):

World Literature ◽

Adenosquamous Carcinoma ◽

Current Treatment ◽

Second Primary ◽

Second Primary Malignancy ◽

Primary Malignancy ◽

The World ◽

Aggressive Tumor ◽

Long Term Prognosis

Adenosquamous carcinoma of the head and neck is a rare and aggressive tumor, with fewer than 100 cases reported in the world literature to date. We report a case of adenosquamous carcinoma of the oral pharynx arising as a second primary malignancy in a patient being treated for primary gastric MALT (mucosa-associated lymphoid tissue) lymphoma. We also review the literature to assess current treatment and long-term prognosis of this rare tumor.

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Long-term prognosis of patients with pediatric pheochromocytoma

Endocrine Related Cancer ◽

10.1530/erc-13-0415 ◽

2013 ◽

Vol 21 (1) ◽

pp. 17-25 ◽

Cited By ~ 58

Author(s):

Birke Bausch ◽

Ulrich Wellner ◽

Dirk Bausch ◽

Francesca Schiavi ◽

Marta Barontini ◽

...

Keyword(s):

Life Expectancy ◽

Malignant Tumors ◽

Germline Mutations ◽

Hereditary Disease ◽

Initial Diagnosis ◽

Second Primary ◽

Term Outcome ◽

Long Term Outcome ◽

Long Term Prognosis

A third of patients with paraganglial tumors, pheochromocytoma, and paraganglioma, carry germline mutations in one of the susceptibility genes, RET, VHL, NF1, SDHAF2, SDHA, SDHB, SDHC, SDHD, TMEM127, and MAX. Despite increasing importance, data for long-term prognosis are scarce in pediatric presentations. The European-American-Pheochromocytoma–Paraganglioma-Registry, with a total of 2001 patients with confirmed paraganglial tumors, was the platform for this study. Molecular genetic and phenotypic classification and assessment of gene-specific long-term outcome with second and/or malignant paraganglial tumors and life expectancy were performed in patients diagnosed at <18 years. Of 177 eligible registrants, 80% had mutations, 49% VHL, 15% SDHB, 10% SDHD, 4% NF1, and one patient each in RET, SDHA, and SDHC. A second primary paraganglial tumor developed in 38% with increasing frequency over time, reaching 50% at 30 years after initial diagnosis. Their prevalence was associated with hereditary disease (P=0.001), particularly in VHL and SDHD mutation carriers (VHL vs others, P=0.001 and SDHD vs others, P=0.042). A total of 16 (9%) patients with hereditary disease had malignant tumors, ten at initial diagnosis and another six during follow-up. The highest prevalence was associated with SDHB (SDHB vs others, P<0.001). Eight patients died (5%), all of whom had germline mutations. Mean life expectancy was 62 years with hereditary disease. Hereditary disease and the underlying germline mutation define the long-term prognosis of pediatric patients in terms of prevalence and time of second primaries, malignant transformation, and survival. Based on these data, gene-adjusted, specific surveillance guidelines can help effective preventive medicine.

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In Acute IgA Nephropathy, Proteinuria and Creatinine Are in the Spot, but Podocyturia Operates in Silence: Any Place for Amiloride?

Case Reports in Nephrology ◽

10.1155/2017/1292531 ◽

2017 ◽

Vol 2017 ◽

pp. 1-4

Author(s):

H. Trimarchi ◽

M. Paulero ◽

R. Canzonieri ◽

A. Schiel ◽

A. Iotti ◽

...

Keyword(s):

Iga Nephropathy ◽

Specific Treatment ◽

Young Male ◽

Normal Subjects ◽

Response To Therapy ◽

Long Term Prognosis ◽

One Year ◽

Stage Renal Disease ◽

End Stage

IgA nephropathy is the most frequent cause of primary glomerulonephritis, portends erratic patterns of clinical presentation, and lacks specific treatment. In general, it slowly progresses to end-stage renal disease. The clinical course and the response to therapy are usually assessed with proteinuria and serum creatinine. Validated biomarkers have not been identified yet. In this report, we present a case of acute renal injury with proteinuria and microscopic hematuria in a young male. A kidney biopsy disclosed IgA nephropathy. Podocyturia was significantly elevated compared to normal subjects. Proteinuria, renal function, and podocyturia improved promptly after steroids and these variables remained normal after one year of follow-up, when steroids had already been discontinued and patient continued on valsartan and amiloride. Our report demonstrates that podocyturia is critically elevated during an acute episode of IgA nephropathy, and its occurrence may explain the grim long-term prognosis of this entity. Whether podocyturia could be employed in IgA nephropathy as a trustable biomarker for treatment assessment or even for early diagnosis of IgA nephropathy relapses should be further investigated.

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