The Relationships between the Clinical Course and Pathologic Features of Poststreptococcal Glomerulonephritis

Aim: To study the relationship between the genotype and the phenotype in the patients with Hermansky — Pudlak syndrome (HPS) associated with granulomatous colitis; to monitor clinical course of the disease for adequate treatment, cancer surveillance and genetic counseling. Materials and Methods: The diagnosis of HPS is established by physical examination, chest X-ray, computed tomography, endoscopic examination with biopsy, and laboratory tests, including histology, baseline laboratory blood, urine and feces tests, determination of ASCA-C and ANCA antibodies using an ELISA. Molecular genetic testing for HPS gene mutations, R702W, G908R, L1007fs and P268S mutations in NOD2 gene, and TaqI variant of the VDR gene were carried out. Results: We report 2 cases of HPS from unrelated families. Both were complicated by inflammatory bowel disease with pathologic features of Crohn’s disease refractory to antibiotics and corticosteroids. One patient (family 1) with Ashkenazi Jewish ancestry had pathogenic variant of the HPS-4 gene in exon 8, mutation P268S of NOD2 genes and “Tt” genotype of TaqI variant of the VDR gene. Another patient (family 2) carried two mutations P268S and G908R of NOD2 gene, and had a large paraovarian cyst diagnosed. No consistent success with the standard medical therapy, used for treating granulomatous colitis, associated with HPS, in presented cases was achieved. Patients needed surgical interventions at a young age and a long-term surveillance of the probable development of tumors and other complications. Azathioprine at 2 mg/kg/day and mesalazine 3 g/day were used with some positive effect for prevention of Crohn’s disease postoperative recurrence. Conclusion: The occurrence of perianal lesions, the histopathological findings and the results of the molecular genetic analysis confirmed the mutations P268S and G908R of NOD2 gene in these cases suggest that HPS was truly associated with Crohn’s disease variant with early onset and severe course. The search for the molecular causes of the disease in some individuals may help in the development of new therapeutic and surgical approaches, as well in the improvement of understanding of premalignant inflammatory conditions in a large bowel.

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Small Renal Neoplasms: Diagnostic Imaging, Pathologic Features, and Clinical Course

Radiology ◽

10.1148/radiology.159.3.817-a ◽

1986 ◽

Vol 159 (3) ◽

pp. 817-817

Author(s):

Errol Levine

Keyword(s):

Diagnostic Imaging ◽

Clinical Course ◽

Renal Neoplasms ◽

Pathologic Features

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Micropapillary Variant of Urothelial Carcinoma in the Upper Urinary Tract: A Clinicopathologic Study of 11 Cases

Archives of Pathology & Laboratory Medicine ◽

10.5858/133.1.62 ◽

2009 ◽

Vol 133 (1) ◽

pp. 62-66 ◽

Cited By ~ 2

Author(s):

Charles C. Guo ◽

Pheroze Tamboli ◽

Bogdan Czerniak

Keyword(s):

Urinary Tract ◽

Urothelial Carcinoma ◽

Lymphovascular Invasion ◽

Clinical Course ◽

Clinical Information ◽

Distant Metastases ◽

Upper Urinary Tract ◽

Clinicopathologic Study ◽

Pathologic Features

Abstract Context.—Micropapillary urothelial carcinoma (MPUC) is a rare variant of urothelial carcinoma. Most studies of MPUC have focused on the urinary bladder, but MPUC of the upper urinary tract remains to be investigated. Objective.—To investigate the pathologic features and clinical significance of MPUC in the upper urinary tract. Design.—We searched the pathology files at our institution and identified 11 cases of MPUC of the upper urinary tract. The histology slides were reviewed, and the clinical information was obtained by review of medical charts. Results.—The average age of the patients was 64.2 years (range, 22–76 years). The tumors were located in the renal pelvis (n = 5), ureter (n = 4), and ureteropelvic junction (n = 2). In all cases, MPUC accounted for an average of 45% (range, 10%–80%) of the tumor and was associated with conventional urothelial carcinoma. Lymphovascular invasion was present in all cases, and metastasis to lymph node was present in 4 of 5 patients whose lymph nodes were dissected. Two patients presented with pT2 disease, and both were alive without evidence of disease at 85 and 119 months after surgery. The other 9 patients presented with pT3 or pT4 disease: 4 of them died of disease at an average of 18 months; 4 surviving patients developed distant metastases; and 1 surviving patient with limited follow-up (6 months) showed no evidence of disease. Conclusions.—Micropapillary urothelial carcinoma of the upper urinary tract often presents at an advanced stage with lymphovascular invasion and distant metastasis. The presence of MPUC, even focal, indicates a poor clinical course.

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Pathologic features and clinical course of a non-functioning primary pulmonary paraganglioma: A case report

Annals of Medicine and Surgery ◽

10.1016/j.amsu.2020.05.027 ◽

2020 ◽

Vol 55 ◽

pp. 185-189

Author(s):

Angélica Tobón ◽

Mauricio Velásquez ◽

Bladimir Pérez ◽

Valeria Zúñiga ◽

Luz F. Sua ◽

...

Keyword(s):

Case Report ◽

Clinical Course ◽

Pathologic Features

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Clinical course and pathologic features of conjunctival non-Hodgkin's lymphoma

Graefe s Archive for Clinical and Experimental Ophthalmology ◽

10.1007/bf00920029 ◽

1990 ◽

Vol 228 (3) ◽

pp. 246-251 ◽

Cited By ~ 6

Author(s):

Hussein A. Khalil ◽

Rob JW de Keizer ◽

Philip M. Kluin ◽

Hanneke C. Kluin-Nelemans ◽

Didi de Wolff-Rouendaal

Keyword(s):

Hodgkin’S Lymphoma ◽

Clinical Course ◽

Hodgkin's Lymphoma ◽

Non Hodgkin’S Lymphoma ◽

Pathologic Features ◽

Non Hodgkin's Lymphoma

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Sacral Chordoma in Early Childhood: Clinicopathological and Immunohistochemical Study

Pediatric and Developmental Pathology ◽

10.1007/s100249900057 ◽

1998 ◽

Vol 1 (5) ◽

pp. 420-426 ◽

Cited By ~ 17

Author(s):

Yoko Iwasa ◽

Yasuaki Nakashima ◽

Hideaki Okajima ◽

Shinobu Morishita

Keyword(s):

Epithelial Membrane Antigen ◽

Immunohistochemical Study ◽

Clinical Course ◽

Initial Diagnosis ◽

Sacral Chordoma ◽

Pathologic Features ◽

S 100 ◽

Aggressive Tumor ◽

Aggressive Clinical Course

Two cases of sacral chordoma in a 7-year, 9-month-old boy and a 3-year, 4-month-old boy are presented. In addition to the typical histology of conventional chordoma, both tumors showed the less differentiated sarcomatoid appearance of atypical chordoma in the major portion. Immunohistochemically, in both cases neoplastic cells in areas of conventional as well as atypical chordoma were positive for keratins (CAM 5.2, AE1 and AE3), epithelial membrane antigen, vimentin, S-100 protein, carcinoembryonic antigen, and glial fibrillary acidic protein. Both patients underwent resection of the tumor and chemotherapy. In comparison with conventional chordomas in adults, however, these two tumors showed more aggressive clinical course and were less amenable to therapeutic control. The older boy died of multiple metastasis 1 year after initial diagnosis. At the last follow-up, 15 months after initial diagnosis, the younger boy was alive, but with recurrent and metastatic disease of the left parasacral area and chest wall. Our studies of these two cases and the reported cases suggest that sacral chordoma in children has distinctive clinico-pathologic features denoting a highly aggressive tumor and that it should be treated as such.

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Prognostic factors and survial of advanced renal cell carcinoma with predominant sarcomatoid histology.

Journal of Clinical Oncology ◽

10.1200/jco.2011.29.7_suppl.400 ◽

2011 ◽

Vol 29 (7_suppl) ◽

pp. 400-400

Author(s):

I. Park ◽

J. Lee ◽

J. Ahn ◽

D. Lee ◽

C. Song ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Prognostic Factors ◽

Cell Carcinoma ◽

Metastatic Disease ◽

Renal Cell ◽

Clinical Course ◽

Performance Status ◽

Sarcomatoid Carcinoma ◽

Response To Treatment ◽

Pathologic Features

400 Background: Sarcomatoid renal cell carcinoma (SRCC) is known to have aggressive clinical course and poor response to treatment. Very limited data of clinical feature, prognostic factor, and survival are available for advanced disease with predominant sarcomatoid histology. We evaluated clinical features, response to treatment, and prognostic factors for survival. Methods: Between 2001 and 2010, 30 patients with metastatic or recurrent RCC with predominant sarcomatoid histology (sarcomatoid component 30% or more for resected kidney or exclusive sarcomatoid carcinoma on needle biopsy) were treated at our institution. We reviewed these patients' records to identify clinical and pathologic features which could affect survival. The role of nephrectomy and systemic therapy on patient outcome was also investigated. Results: There were 20 male and 10 female patients with a median age of 58 years (range, 43–83). Twenty patients had initially metastatic disease and 16 patients (53%) had ECOG performance status (PS) of 0–1. The most frequent metastatic site was lung (57%) followed by bone (43%) and distant lymph nodes (23%). Fourteen (70%) out of 20 patients with initially metastatic disease underwent primary nephrectomy and six patients also underwent metastasectomy. The median % of sarcomatoid component was 80% (range, 30–100%). All patients (N=10) who received immunotherapy had progressive disease as their best response and only one out of 5 patients treated with sunitinib or everolimus had a partial response. With a median follow-up duration of 22 months, the median survival was 3.6 months (95% CI, 0∼7.5) with 6-month survival rate of 43%. Only ECOG PS had impact on survival (P<0.001: ECOG=0, 14.1 months; ECOG=1, 7.4 months; ECOG=2, 3.2 months, and ECOG=3, 1.64 months). Survivals for initially metastatic disease were not significantly different whether patients underwent nephrectomy or not (2.0 months vs. 3.4 months, HR=0.62, 95% CI, 0.16–2.44 after correcting for potential prognostic factors). Conclusions: Patients with SRCC have a fulminant clinical course and the majority of patients had disease progression irrespective of any treatment. Only performance status dose have impact on overall survival. No significant financial relationships to disclose.

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Aggressive Variant of a Solid Pseudopapillary Neoplasm: A Case Report and Literature Review

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2013-0184-cr ◽

2014 ◽

Vol 138 (7) ◽

pp. 974-978 ◽

Cited By ~ 15

Author(s):

Bailey A. Reindl ◽

Douglas W. Lynch ◽

Ali D. Jassim

Keyword(s):

Adolescent Girl ◽

Cellular Differentiation ◽

Clinical Course ◽

Solid Pseudopapillary Neoplasm ◽

Proliferative Index ◽

Young Females ◽

Pathologic Features ◽

Extensive Necrosis ◽

Aggressive Variant ◽

Solid Pseudopapillary Neoplasms

Solid pseudopapillary neoplasm, a lesion of uncertain cellular differentiation, is an unusual tumor of the pancreas with an indolent clinical course that typically arises in young females. We report a case of solid pseudopapillary neoplasm arising in a 17-year-old adolescent girl who presented with progressive abdominal pain. The patient underwent surgical resection of an 18 × 14 × 8-cm pancreatic mass that displayed the usual histologic features of a solid pseudopapillary neoplasm in addition to prominent nuclear atypia, increased proliferative index, and extensive necrosis. These unusual histologic findings are rare and are of particular interest owing to the dramatically decreased survival time displayed in this case. Although precise pathologic criteria suggesting a high risk for aggressive behavior of solid pseudopapillary neoplasms are uncertain, recognition of the unusual pathologic features displayed in this case may be useful in the prediction of potentially more aggressive neoplasms that portend a poorer prognosis.

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Necrotizing Meningoencephalitis of Maltese Dogs

Veterinary Pathology ◽

10.1177/030098589503200303 ◽

1995 ◽

Vol 32 (3) ◽

pp. 230-235 ◽

Cited By ~ 66

Author(s):

I. H. Stalis ◽

B. Chadwick ◽

B. Dayrell-Hart ◽

B. A. Summers ◽

T. J. Van Winkle

Keyword(s):

Cerebrospinal Fluid ◽

White Matter ◽

Clinical Course ◽

Clinical Signs ◽

Lateral Ventricles ◽

Pathologic Features ◽

Cerebrospinal Fluid Examination ◽

History Of ◽

Extensive Necrosis

The clinical and pathologic features of five young Maltese dogs with a necrotizing meningoencephalitis were studied and compared with published reports of the necrotizing meningoencephalitis of Pug dogs. The ages of the Maltese dogs ranged from 9 months to 4 years. Four dogs were male, and one was female. The dogs had a history of seizures with or without other neurologic signs for 3 days to 20 weeks prior to death. Cerebrospinal fluid examination in three dogs revealed a pleocytosis and elevated levels of protein. At necropsy, the cerebrum was asymmetrically swollen in four dogs, with a loss of distinction between the gray and white matter and mild to moderate asymmetrical dilation of the lateral ventricles. Histologically, there was extensive necrosis and nonsuppurative inflammation of the cerebral gray and white matter, overlying meninges, and adjacent thalamus and hippocampus. The 4-year-old dog had the longest duration of clinical signs and had little inflammation but extensive atrophy of affected areas, with astrocytosis. The clinical course and pathologic changes in these Maltese dogs are indistinguishable from those in reported cases of necrotizing meningoencephalitis of Pug dogs, indicating that this lesion is probably not unique to Pug dogs.

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