scholarly journals A Solitary Intestinal Myofibroma: A Rare Cause of Neonatal Anemia

2017 ◽  
Vol 10 (3) ◽  
pp. 890-896 ◽  
Author(s):  
Janna S.E. Ottenhoff ◽  
Peter G.J. Nikkels ◽  
C.E.J. Terwisscha van Scheltinga ◽  
L. Naeije
Keyword(s):  
Poor Prognosis ◽  
Unusual Case ◽  
Survival Rates ◽  
Multiple Form ◽  
Total Resection ◽  
Solitary Lesion ◽  
Total Body ◽  
Lesion Treatment ◽  
Infantile Myofibroma ◽  
Rule Out

Solitary infantile myofibroma with visceral involvement is very rare. We present an unusual case of a solitary myofibroma with abdominal localization in a 1-day-old female neonate who presented with severe anemia and rectal bleeding. A bleeding myofibroma was found, located in the wall of the jejunum, and totally resected. In case of a solitary lesion, treatment is relatively easy and effective, with excellent prognosis after total resection. The multiple form (myofibromatosis) has a poor prognosis with low survival rates. We therefore recommend total body MRI for all patients diagnosed with myofibroma to rule out other lesions.

Evolution of Surgical Approaches in the Treatment of Petroclival Meningiomas: A Retrospective Review

2007 ◽  
Vol 61 (suppl_5) ◽  
pp. ONS202-ONS211 ◽  
Author(s):  
Nicholas C. Bambakidis ◽  
U. Kumar Kakarla ◽  
Louis J. Kim ◽  
Peter Nakaji ◽  
Randall W. Porter ◽  
...  
Keyword(s):  
Survival Rates ◽  
Progression Free Survival ◽  
Surgical Approaches ◽  
Single Institution ◽  
Short Term ◽  
Total Resection ◽  
Free Survival ◽  
Petroclival Meningioma ◽  
Petroclival Meningiomas

Abstract Objective: We examined the surgical approaches used at a single institution to treat petroclival meningioma and evaluated changes in method utilization over time. Methods: Craniotomies performed to treat petroclival meningioma between September of 1994 and July of 2005 were examined retrospectively. We reviewed 46 patients (mean follow-up, 3.6 yr). Techniques included combined petrosal or transcochlear approaches (15% of patients), retrosigmoid craniotomies with or without some degree of petrosectomy (59% of patients), orbitozygomatic craniotomies (7% of patients), and combined orbitozygomatic-retrosigmoid approaches (19% of patients). In 18 patients, the tumor extended supratentorially. Overall, the rate of gross total resection was 43%. Seven patients demonstrated progression over a mean of 5.9 years. No patients died. At 36 months, the progression-free survival rate for patients treated without petrosal approaches was 96%. Of 14 patients treated with stereotactic radiosurgery, none developed progression. Conclusion: Over the study period, a diminishing proportion of patients with petroclival meningioma were treated using petrosal approaches. Utilization of the orbitozygomatic and retrosigmoid approaches alone or in combination provided a viable alternative to petrosal approaches for treatment of petroclival meningioma. Regardless of approach, progression-free survival rates were excellent over short-term follow-up period.

An Unusual Case of Desmoplastic Melanoma With Monster Cells Imitating an Atypical Fibroxanthoma

2021 ◽  
pp. 106689692110447
Author(s):  
Juan J. Ríos-Martín ◽  
Manuel Pérez-Pérez ◽  
Sebastián Umbría-Jiménez ◽  
David Moreno-Ramírez ◽  
Ana Vallejo-Benítez
Keyword(s):  
Cyclin D1 ◽  
Copy Number ◽  
Poor Prognosis ◽  
Unusual Case ◽  
Prognostic Significance ◽  
Mutation Status ◽  
Atypical Fibroxanthoma ◽  
Desmoplastic Melanoma ◽  
Aggressive Tumor

Numerous cells with very large and irregular nuclei (“monster” cells) have not hitherto been reported in desmoplastic melanoma (DM). Their prognostic significance in melanomas is a matter of debate, although some authors have associated them with more aggressive tumor behavior. We report a mixed DM on the scalp of an 88-year-old woman imitating an atypical fibroxanthoma. Tumor cells stained positive for SOX10, S100, and cyclin D1; BRAF mutation status was negative, and fluorescence in situ hybridization analysis showed copy number gains in 11q13 (cyclin D1) and 6p25 (RREB1), and loss in 6q23 (MYB). Cyclin D1 amplification is associated with poor prognosis in melanoma.

scholarly journals Multi-modal Ultrasonic Diagnosis and Prognosis for Primary Thyroid Lymphoma Invasiveness

2021 ◽  
Author(s):  
Chenjuan Peng ◽  
Chen Yang ◽  
Jincao Yao ◽  
LingYan Zhou ◽  
Jingjing Xu ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Survival Rates ◽  
Diagnostic Value ◽  
Thyroid Lymphoma ◽  
Diagnosis And Prognosis ◽  
Kaplan Meier ◽  
Primary Thyroid Lymphoma ◽  
Ultrasonic Images ◽  
Mixed Types ◽  
Thyroid Capsule

Abstract Objective Sixty-nine patients with primary thyroid lymphoma (PTL) were evaluated for diagnostic value and prognosis for PTL invasiveness. Methods We retrospectively (2008–2019) analyzed multi-modal ultrasonic images and clinical characteristics from pathologically confirmed PTL patients. These patients were divided into aggressive PTL(n=46) and indolent PTL(n=23). Results Age(>70 years old) and elevated LDH (lactase dehydrogenase) were statistically different clinical features between aggressive and indolent PTL. From ultrasonic images, 34 cases were nodular, 11 diffused, and 24 mixed. Mixed types displayed high invasiveness (45.7%) while diffuse types displayed higher inertness (39.1%), and differences were statistically significant (P = 0.000). Elastography, invaded thyroid capsule and increased chaotic vascularity also showed significant differences between aggressive and indolent PTL. We observed statistical difference in OS(overall survival rates) between aggressive and indolent PTL(p=0.032). Single factor Kaplan-Meier (K-M) analysis showed that: age > 70 years old, aggressive pathology, Ki67>30%, elastography scored >3 were positively correlated with the risk of poor prognosis of PTL (P < 0.05).Conclusions Multi-modal ultrasound provides accurate ultrasonographic information, e.g., ultrasound patterns, elastography, invaded thyroid capsules, and hypervascularity, which facilitates PTL invasiveness diagnostics for improved clinical treatment. In addition, PTL patients with age > 70 years old, aggressive pathology, Ki67>30%, elastography scored >3 are more likely to have poor prognosis.

scholarly journals Treatment-Resistant Hypercalcemia Secondary to Ectopic PTH Hypersecretion From Disseminated Ovarian Cancer

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A228-A229
Author(s):  
Alexander M Balinski ◽  
Neil J Khatter ◽  
Jeffrey M Gold ◽  
Krishna S Pothugunta ◽  
Vamshi K Garlapaty ◽  
...  
Keyword(s):  
Ovarian Cancer ◽  
Early Detection ◽  
Serum Calcium ◽  
Poor Prognosis ◽  
Malignant Tumors ◽  
Ovarian Adenocarcinoma ◽  
Hypercalcemia Of Malignancy ◽  
Organ System Failure ◽  
Sestamibi Scan ◽  
Total Body

Abstract Background: Hypercalcemia of malignancy (HCM) can present secondary to hypersecretion of parathyroid hormone (PTH)-related protein (PTHrP) from malignant tumors, but rare cases of HCM have also been documented due to inappropriate PTH secretion from ectopic neoplasms. Here, we report an unusual case of HCM due to hypersecretion of PTH from a disseminated mucinous ovarian adenocarcinoma. Case Presentation: A 45-year-old female presented with confusion, constipation, fatigue, and abdominal pain two weeks after total abdominal hysterectomy with bilateral salpingo-oophorectomy and suboptimal debulking of a newly discovered left ovarian mucinous adenocarcinoma with metastasis to the bladder, parametrium, vagina, right ovary, and rectosigmoid. Subsequent CT revealed numerous bilateral pulmonary nodules, hilar adenopathy, liver lesions, and abdominal adenopathy. On exam, she was tachycardic and hypertensive with diaphoresis, dry mucous membranes, respiratory distress, guarded abdominal tenderness, and altered mental status. Her labs were significant for a serum calcium of 21.7 mg/dL, creatinine of 1.93 mg/dL, ferritin of 2,379 ng/mL, leukocytosis of 21.9 bil/L, PTH of 1,061 pg/mL, and PTHrP of 29 pmol/L. Ectopic PTH secretion was highly suspected after negative parathyroid ultrasound. Pamidronate (60 mg IV), calcitonin (200 U IM), and fluid resuscitation were unable to normalize her serum calcium, resulting in the need for dialysis and subsequent continuous renal replacement therapy. Further intervention with denosumab (120 mg SQ), etelcalcetide (5 mg IV), and cinacalcet (60 mg PO) was also attempted. Serum calcium began to decline, but repeat PTH resulted greater than 2,500 pg/mL. Unfortunately, the patient died just one week into her hospital course from septic shock and multi-organ system failure. Discussion: Hypercalcemia of malignancy typically arises from tumor secretion of PTHrP, cytokine release from osteolytic metastases, or tumor production of calcitriol. In cases of hypercalcemia due to excess PTH secretion, primary parathyroid etiologies are typically considered while ectopic PTH-secreting tumors are rare. PTH staining of biopsy specimens and total body sestamibi scan may prove useful in the early detection and treatment of these tumors, but HCM offers a poor prognosis with mean survival of 2 to 3 months and in-hospital mortality of 6.8%. Currently, there are only three cases in the reported literature of ectopic PTH-induced hypercalcemia related to ovarian cancer. To our knowledge, this is the fourth reported case. Conclusion: Ectopic PTH-secreting tumors carry a poor prognosis and should be considered in cancer patients presenting with PTH-associated hypercalcemia. Biopsy staining for PTH and total body sestamibi scan may assist in the early detection of these tumors, but current treatment strategies offer suboptimal outcomes.

Multiple Isolated Extramedullary Relapse and Long-term Survival of One AML Patient

2020 ◽  
Author(s):  
Jiman Li ◽  
Yang Liu ◽  
Yunzhu Li ◽  
liu weiping
Keyword(s):  
Complete Remission ◽  
Poor Prognosis ◽  
Unusual Case ◽  
Granulocytic Sarcoma ◽  
Accurate Diagnosis ◽  
Physical Damage ◽  
Term Survival ◽  
Case Presentation ◽  
Extramedullary Relapse

Abstract Background: Granulocytic sarcomas (GS) are very rare. If it occurs after complete remission of acute myeloblastic leukemia（AML）, it indicates a recurrence of AML and a poor prognosis. In such cases, relapse of leukemia occurs within a mean of 10 months following granulocytic sarcoma.Case presentation: Here we present an unusual case of 78-year-old male who presented with AML-M1 38 years ago. After complete remission from AML-M1 6 years later, he developed unusual multiple isolated extramedullary relapses. And the extramedullary relapse occurred 7 times and involved 8 anatomic sites during 15 years. Despite repeated relapses, treatment and physical damage, the patient managed to survive into 2016.However, we did not detect any signs of leukemia after 1992 and his bone marrow and peripheral blood remained normal Until his death. Immunohistochemical results of our case are all the same, suggesting that they were all derived from the recurrence of the same tumor.Conclusions: Extramedullary relapses may occur in AML patients after complete relieve and without the blood count and BM involvement. Accurate diagnosis of GS is important so the patient could to be treated timely. It is a challenge for the pathologist to make the diagnosis, and without immunohistochemistry (IHC), it may be misdiagnosed as another tumor.

LMCE3 Treatment Strategy: Results in 99 Consecutively Diagnosed Stage 4 Neuroblastomas in Children Older Than 1 Year at Diagnosis

2000 ◽  
Vol 18 (3) ◽  
pp. 468-468 ◽  
Author(s):  
D. Frappaz ◽  
J. Michon ◽  
C. Coze ◽  
C. Berger ◽  
E. Plouvier ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Complete Remission ◽  
Survival Rates ◽  
Progression Free Survival ◽  
Consolidation Therapy ◽  
French Society ◽  
Free Survival ◽  
Stage 4 ◽  
Total Body ◽  
Better Than

PURPOSE: To tailor postinduction therapy for stage 4 neuroblastoma in children who are older than 1 year at diagnosis according to status after induction. PATIENTS AND METHODS: From March 1987 to December 1992, 99 patients who were consecutively admitted were included in the Lyon-Marseille-Curie East of France (LMCE)3 strategy. After induction with the French Society of Pediatric Oncology NB87 regimen and surgery, patients who were in complete remission immediately proceeded to consolidation therapy with vincristine, melphalan, and fractionated total-body irradiation (VMT). All other patients underwent a postinduction strategy before VMT, either an additional megatherapy regimen or further chemotherapy with etoposide/carboplatin. RESULTS: The progression-free survival (PFS) is 29% at 7 years from diagnosis, which compares favorably with that of a similar cohort of 72 patients previously reported by our group (LMCE1; PFS of 20% at 5 years and 8% at 14 years, P = .004). In the multivariate analysis, only age younger than 3 years at diagnosis (P = .0085) and achievement of complete or very good partial remission after NB87 and surgery (P = .00024) remained significant. The PFS of the 87 patients who were included in the postinduction strategy was significantly better than that of the comparable 62 patients on the LMCE1 study (32% v 11% at 7 years; P = .005). CONCLUSION: The progressive improvements in the LMCE results over the last 10 years suggest that improvements in supportive care measures and increases in each component of this strategy (induction, postinduction, consolidation) may all contribute to increased survival rates.

Factors affecting outcome following treatment of patients with cavernous sinus meningiomas

2010 ◽  
Vol 113 (5) ◽  
pp. 1087-1092 ◽  
Author(s):  
Michael E. Sughrue ◽  
Martin J. Rutkowski ◽  
Derick Aranda ◽  
Igor J. Barani ◽  
Michael W. McDermott ◽  
...  
Keyword(s):  
Cavernous Sinus ◽  
Postoperative Radiotherapy ◽  
Meta Analysis ◽  
Survival Rates ◽  
Extent Of Resection ◽  
Gross Total Resection ◽  
Cranial Neuropathy ◽  
Tumor Control ◽  
Subtotal Resection ◽  
Total Resection

Object Although there is a considerable volume of literature available on the treatment of patients with cavernous sinus meningiomas (CSMs), most of the data regarding tumor control and survival come from case studies or single-institution series. The authors performed a meta-analysis of reported tumor control and survival rates of patients described in the published literature, with an emphasis on specific prognostic factors. Methods The authors systematically analyzed the published literature and found more than 3000 patients treated for CSMs. Separate meta-analyses were performed to calculate pooled rates of recurrence and cranial neuropathy after 1) gross-total resection, 2) subtotal resection without adjuvant postoperative radiotherapy or radiosurgery, and 3) stereotactic radiosurgery (SRS) alone. Results were expressed as pooled proportions, and random-effects models were used to incorporate any heterogeneity present to generate a pooled proportion. Individual studies were weighted using the inverse variance method, and 95% CIs for each group were calculated from the pooled proportions. Results A total of 2065 nonduplicated patients treated for CSM met inclusion criteria for the analysis. Comparisons of the 95% CIs for recurrence of these 3 cohorts revealed that SRS-treated patients experienced improved rates of recurrence (3.2% [95% CI 1.9–4.5%]) compared with either gross-total resection (11.8% [95% CI 7.4–16.1%]) or subtotal resection alone (11.1% [95% CI 6.6–15.7%]) (p < 0.01). The authors found that the pooled mixed-effects rate of cranial neuropathy was markedly higher in patients undergoing resection (59.6% [95% CI 50.3–67.5%]) than for those undergoing SRS alone (25.7% [95% CI 11.5–38.9%]) (p < 0.05). Conclusions Radiosurgery provided improved rates of tumor control compared with surgery alone, regardless of the subjective extent of resection.

Rosai–Dorfman disease of multiple organs, including the epicardium: An unusual case with poor prognosis

Heart & Lung ◽  
2011 ◽  
Vol 40 (2) ◽  
pp. 168-171 ◽  
Author(s):  
Jiquan Chen ◽  
Hao Tang ◽  
Bing Li ◽  
Qingyu Xiu
Keyword(s):  
Poor Prognosis ◽  
Unusual Case ◽  
Multiple Organs ◽  
Rosai Dorfman Disease

Dural Melanoma Associated with Ocular Melanosis and Multiple Blue Nevi

2001 ◽  
Vol 5 (5) ◽  
pp. 381-385 ◽  
Author(s):  
Jason K. Rivers ◽  
Shelly Bhayana ◽  
Magda Martinka
Keyword(s):  
Central Nervous System ◽  
Poor Prognosis ◽  
Surgical Intervention ◽  
Asian Woman ◽  
Total Resection ◽  
Aggressive Disease ◽  
Nevus Of Ota ◽  
The Central Nervous System ◽  
Aggressive Tumors ◽  
Surgical Extirpation

Background: Primary meningeal melanomas of the central nervous system (CNS) are a rare malignant process with the majority originating from the leptomeninges. Primary dural melanomas have been reported to occur in isolation or in conjunction with Nevus of Ota. The association of primary dural melanoma with multiple cutaneous blue nevi has not been reported previously. Objective: To describe a case of a 41-year-old Asian woman patient with a primary dural melanoma that arose in association with ocular melanosis and multiple cutaneous blue nevi. The patient is alive almost more than 8 years after subtotal and subsequent total resection of her primary tumor. Primary dural melanomas, Nevus of Ota, and blue nevi are discussed in relation to their coexistence and potential for intracranial melanoma. Conclusion: CNS melanoma is regarded as an extremely aggressive disease with poor prognosis. This case and previous reports of dural melanomas occurring in isolation or with Nevus of Ota have demonstrated relatively prolonged survival after surgical intervention. We conclude that dural melanomas are less aggressive tumors requiring surgical extirpation only.

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