RARE-31. TREATMENT OUTCOME OF CHILDREN WITH PLEOMORPHIC XANTHOASTROCYTOMA: RETROSPECTIVE ANALYSIS – A SINGLE INSTITUTION EXPERIENCE

Abstract BACKGROUND Pleomorphic xanthoastrocytoma (PXA) and anaplastic pleomorphic xanthoastrocytoma (APXA) are two types of rare astrocytomas in pediatrics. There is limited literature reporting their outcomes. METHODS A retrospective analysis of pediatric patients with diagnosis of PXA and APXA treated at Nicklaus Children’s Hospital was conducted using descriptive measures. RESULTS A chart review of patients with brain tumors from 2001 to 2019 revealed that 13 patients (median age 6 years, range 2–17 years) were diagnosed with non-metastatic PXA (n=12) and APXA (n=1). Six patients (46%) were male. Clinical presentation included seizures (n=8), headaches (n=2) cranial nerve palsies (n=2). Diagnostic imaging showed tumor in the temporal lobe (n=5), parietal lobe (n=2), temporoparietal lobe (n=2), frontal lobe (n=1), occipital lobe (n=1), others (n=2). BRAF V600E mutation were identified in three of five analyzed tumors including PXA (n=4) and APXA (n=1). Surgical intervention consisted of gross total resection (n=8) and near/subtotal resection (n=5). Ten patients (76%) required a second surgery (median 2; range 1–4) due to local recurrence. The median time to recurrence was 3 years (range 6 months - 7 years). Three patients (23%) had malignant transformation of PXA, which occurred three to eight years from the initial surgery; one of them also had extensive spinal metastasis. Focal radiation was given to six patients (46%) due to multiple recurrence (n=2), malignant transformation (n=3), APXA (n=1). At a median follow up of 8 years (range 3 to 20 years), eight patients remain alive, three patients had lost to follow up, two patients died from progressive disease. Long-term sequelae varied as follows; seizures (n=5), motor weakness (n=4), cranial nerve palsies (n=2), and learning disability (n=1). CONCLUSION PXA is associated with high local recurrence rate and uncommonly malignant transformation to APXA in pediatrics, which requires close follow-up due to unpredictable biological behavior of these tumors.

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Recurrent Solitary Fibrous Tumor of the Pleura With Malignant Transformation

Archives of Pathology & Laboratory Medicine ◽

10.5858/2004-128-460-rsftot ◽

2004 ◽

Vol 128 (4) ◽

pp. 460-462

Author(s):

Rani Kanthan ◽

Bahman Torkian

Keyword(s):

Malignant Transformation ◽

Solitary Fibrous Tumor ◽

Immunohistochemical Staining ◽

White Woman ◽

Accurate Diagnosis ◽

Malignant Progression ◽

Biological Behavior ◽

Fibrous Tumor

Abstract Recurrent solitary fibrous tumor of the pleura with malignant progression occurs rarely. We report a case of solitary fibrous tumor of the pleura in an 85-year-old white woman that recurred 4 times during a span of 10 years and subsequently underwent malignant transformation. The accurate diagnosis of solitary fibrous tumor is aided by ancillary techniques, such as immunohistochemical staining; however, with malignant transformation, such tools may be of limited value. Long-term clinical follow-up is recommended for all patients with solitary fibrous tumor because of the potential adverse biological behavior of this tumor, which may lead to repeated recurrences and/or malignant transformation.

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Delayed Cranial Nerve Palsy After Coiling of Carotid Cavernous Sinus Aneurysms

Neurosurgery ◽

10.1227/01.neu.0000369194.13994.62 ◽

2010 ◽

Vol 66 (6) ◽

pp. E1215-E1216 ◽

Cited By ~ 11

Author(s):

David S. Xu ◽

Michael C. Hurley ◽

H. Hunt Batjer ◽

Bernard R. Bendok

Keyword(s):

Cavernous Sinus ◽

Cranial Nerve ◽

Clinical Presentation ◽

Cranial Neuropathy ◽

Aneurysm Neck ◽

Post Procedure ◽

Cranial Nerve Palsies ◽

Endovascular Coils ◽

Healthy Females

Abstract OBJECTIVE Detachable endovascular coils have become a common treatment strategy for carotid cavernous sinus aneurysms (CCAs), but previously unrecognized postprocedure complications may emerge as longer follow-up data are accumulated. In this report, the authors document the first known cases of delayed cranial neuropathy following CCA coiling in 3 patients, all of whom present at least a year postprocedure without aneurysm regrowth. The potential mechanisms underlying this syndrome are discussed as well as their implications on the selection and optimal endovascular management of CCA patients. CLINICAL PRESENTATION Three previously healthy females aged 50, 60, and 62 underwent CCA coiling at our institution and subsequently developed ipsilateral cranial nerve palsies at 56, 28, and 14 months, respectively, post-procedure. At presentation, all 3 patients had a new, recurrent area of flow in their CCA without changes in aneurysm size. INTERVENTION One patient declined further treatment. In the other 2 patients, a stent was placed across the aneurysm neck, and one patient underwent additional coiling. Unfortunately, all 3 patients remained symptomatic at their latest follow-up. Conclusion Because of the intimate anatomic environment of the cavernous sinus, neural elements within it may be particularly susceptible to persistent mass or dynamic effects exacerbated by remnant or recurrent flow across the neck of a coiled aneurysm. These 3 cases prompted the authors to advocate for more aggressive efforts to achieve and maintain CCA occlusion. Furthermore, when such efforts are unsuccessful, consideration of traditional carotid occlusion strategies with or without bypass is warranted.

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Malignant Transformation of Nodular Hidradenoma in the Lower Leg

Case Reports in Oncology ◽

10.1159/000489255 ◽

2018 ◽

Vol 11 (2) ◽

pp. 298-304

Author(s):

Nhuan Ngo ◽

Michiro Susa ◽

Takahiro Nakagawa ◽

Yo Kawahara ◽

Chiaki Sato ◽

...

Keyword(s):

Local Recurrence ◽

Malignant Transformation ◽

Rare Case ◽

Lower Leg ◽

Sweat Glands ◽

Anterior Surface ◽

Adnexal Tumor ◽

Nodular Hidradenoma

Nodular hidradenoma (NH) is a benign adnexal tumor that arises from either eccrine or apocrine sweat glands. NH can originate from any cutaneous site, but the most common sites are the head and anterior surface of the trunk, with very rare cases in the extremities. Long-standing NH has been reported to undergo malignant transformation to malignant NH (MNH); however, its occurrence in the lower leg is extremely rare with only one other case reported to date. In this report, we present a rare case of MNH occurring in the lower leg which was resected with the intent to make a diagnosis. At the final follow-up after 11 months, no local recurrence or metastasis has been observed.

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Microneurosurgical Treatment of Intracranial Dermoid and Epidermoid Tumors

Neurosurgery ◽

10.1227/00006123-198904000-00012 ◽

1989 ◽

Vol 24 (4) ◽

pp. 561-567 ◽

Cited By ~ 203

Author(s):

M. Gazi Yaşargil ◽

Chad D. Abernathey ◽

Ali ç. Sarioglu

Keyword(s):

Cranial Nerve ◽

Relaxation Times ◽

T2 Relaxation ◽

Female Ratio ◽

Clinical Presentations ◽

Cranial Nerve Palsies ◽

Dermoid Tumor ◽

Similar Appearance ◽

Male To Female

ABSTRACT Forty-three patients with intracranial, intradural dermoid (8) and epidermoid (35) tumors underwent radical surgical resection utilizing strict microneurosurgical technique. The average age was 37.3 years for the patients with epidermoid tumors and 36.2 years for the patients with dermoid tumors. The male to female ratio was 3:2 for the epidermoid group and 3:1 for the dermoid group. Common clinical presentations included cerebellar dysfunction, cranial nerve impairment, and seizures. Typically, computed tomography scans revealed the epidermoid tumors (30 cases studied) as nonhomogeneous hypodense lesions with irregular borders and without contrast enhancement. The dermoid tumors (7 cases studied) had a similar appearance, but with a wider range of attenuation values. Magnetic resonance imaging findings for the epidermoid tumors (6 cases studied) consisted of increased T1 and increased T2 relaxation times. Supratentorial tumors were excised by the pterional (frontosphenotemporal) approach, mesencephalic tumors by either a supratentorial posterior interhemispheric transtentorial approach or an infratentorial/supracerebellar method, and posterior fossa tumors by either a medially or laterally positioned suboccipital osteoplastic craniotomy. One epidermoid tumor and one dermoid tumor were considered to be subtotally resected because of dense adherences left attached to vital structures; the remaining 41 tumors were completely excised. The most frequent complications were aseptic/chemical meningitis and transient cranial nerve palsies. There were no perioperative deaths. Mean follow-up was 5.2 years. Eighty-six percent of patients reported good to excellent results. No patient had experienced symptomatic or radiographic evidence of recurrence. These results suggest that although dermoid and epidermoid tumors tend to cross anatomical boundaries via the subarachnoid system, in the majority of patients tumors can be resected in their entirety with relatively low morbidity by utilizing microneurosurgical techniques.

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Pilomatrixoma with Atypical Features: A Case Report

Case Reports in Dermatology ◽

10.1159/000511696 ◽

2021 ◽

pp. 98-102

Author(s):

Meina Missak ◽

Aaron Haig ◽

Manal Gabril

Keyword(s):

Case Report ◽

Malignant Transformation ◽

Mitotic Rate ◽

High Power Field ◽

Atypical Features ◽

High Local Recurrence Rate ◽

Hair Matrix ◽

Malignant Changes ◽

Pilomatrix Carcinoma

Pilomatrixoma is an uncommon, benign tumor with differentiation towards both the hair matrix and cells arising in the cortex, most frequently appearing in the first or second decade of life. In rare instances, pilomatrixomas can show malignant transformation. Pilomatrix carcinoma is extremely uncommon and has traditionally been considered a tumor of low malignant potential; however, a high local recurrence rate has been reported. There is a paucity of literature on these lesions, with only a few reports describing the spectrum of malignant changes seen in these lesions. In this case report, we present a case of pilomatrixoma in an adult patient showing atypical features. While the tumor is small, there are focal features that suggest progression to malignancy, but do not fulfill the criteria for pilomatrix carcinoma. These focal atypical features include a focal infiltrative pattern at the periphery, with a variable cytological atypia and an increased mitotic rate, up to five mitotic events/high-power field. Irregular foci of central necrosis (comedonecrosis) were present in several lobules. Some of the features identified were similar to a subset of pilomatrixoma, known as “proliferating pilomatrixoma.” However, our case did not have the diffuse changes or larger size that has been frequently reported in “proliferating pilomatrixoma.” In conclusion, given the lack of focality of the changes, the lesion in our case is best described as a pilomatricoma with atypical features. Furthermore, our case may highlight the need to ensure close clinical follow-up for these lesions with unexpected atypical features that raise concern of recurrence and malignant transformation.

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Simple Mucinous Cyst of the Pancreas: Review and Update

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2017-0232-ra ◽

2017 ◽

Vol 141 (10) ◽

pp. 1330-1335 ◽

Cited By ~ 3

Author(s):

Shula Schechter ◽

Jiaqi Shi

Keyword(s):

Malignant Transformation ◽

Current Knowledge ◽

Biological Behavior ◽

Pancreatic Cysts ◽

Molecular Characteristics ◽

Practice Experience ◽

Gastric Type ◽

Neoplastic Process ◽

Mucinous Cyst

Context.— According to the 2014 Baltimore Consensus Meeting, simple mucinous cysts are defined as macroscopic cysts that are greater than 1 cm in size with gastric-type flat mucinous lining and minimal cytologic atypia without ovarian-type stroma. This lesion has not been widely recognized and has undergone a recent nomenclature change owing to unclear pathogenesis and biologic behavior. Mucinous pancreatic cystic lesions are generally considered precursor lesions of pancreatic adenocarcinoma. However, simple mucinous cysts generally have benign behavior with no recurrence or malignant transformation during short follow-up periods. Objective.— To provide a brief update and summary of the evolving nomenclature and current knowledge of simple mucinous cysts with an overview of their clinical, histopathologic, immunohistochemical, and molecular characteristics, as well as discussion of their differential diagnoses and biological behavior. Data Sources.— Analysis of the pertinent literature (PubMed) and authors' clinical practice experience based on institutional and consultation materials. Conclusions.— Simple mucinous cyst has undergone a nomenclature evolution from mucinous nonneoplastic cyst to the current recommended name of simple mucinous cyst to reflect its unclear pathogenesis and progression. The malignant potential of simple mucinous cyst is still debatable. Recent molecular studies support a neoplastic process, but these cysts generally exhibit benign behavior without recurrence or malignant transformation. Therefore, accurate diagnosis of simple mucinous cysts is critical to distinguish them from other more common and aggressive mucinous pancreatic cysts. Studies with larger cohorts and longer clinical follow-up data are needed to further determine the biologic behavior of this cyst and implications for prognosis.

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Sphenoid Sinus Mucocoele and Cranial Nerve Palsies in a Patient with a History of Nasopharyngeal Carcinoma: May Mimic Local Recurrence

Clinical Oncology ◽

10.1053/clon.2001.9288 ◽

2001 ◽

Vol 13 (5) ◽

pp. 353-355

Author(s):

C.S.F. Wong ◽

S.H. Luk ◽

T.W. Leung ◽

K.K. Yuen ◽

W.K. Sze ◽

...

Keyword(s):

Nasopharyngeal Carcinoma ◽

Local Recurrence ◽

Cranial Nerve ◽

Sphenoid Sinus ◽

Cranial Nerve Palsies ◽

History Of

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Sphenoid Sinus Mucocoele and Cranial Nerve Palsies in a Patient with a History of Nasopharyngeal Carcinoma: May Mimic Local Recurrence

Clinical Oncology ◽

10.1007/s001740170034 ◽

2001 ◽

Vol 13 (5) ◽

pp. 353-355

Author(s):

C. S. F. Wong ◽

S. H. Luk ◽

T. W. Leung ◽

K. K. Yuen ◽

W. K. Sze ◽

...

Keyword(s):

Nasopharyngeal Carcinoma ◽

Local Recurrence ◽

Cranial Nerve ◽

Sphenoid Sinus ◽

Cranial Nerve Palsies ◽

History Of

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Inflammatory myofibroblastic tumours of the respiratory system and the impact of the varying patterns

Annals of The Royal College of Surgeons of England ◽

10.1308/rcsann.2016.0033 ◽

2016 ◽

Vol 98 (1) ◽

pp. e8-e10 ◽

Cited By ~ 1

Author(s):

JV Lodhia ◽

TD Christensen ◽

SE Trotter ◽

ES Bishay

Keyword(s):

Local Recurrence ◽

Malignant Transformation ◽

Respiratory System ◽

Surgical Procedure ◽

Complete Resection ◽

Anatomical Location ◽

The Impact

Inflammatory fibroblastic tumours are very rare. They are mostly located in the respiratory system. We present three cases of patients with fibroblastic tumours. The diversity of inflammatory fibroblastic tumours in the respiratory system and the surgical considerations are discussed. Our recommendation is that treatment should include a complete resection to prevent local recurrence and malignant transformation, and follow-up review should reflect the procedure carried out, especially in terms of the anatomical location and the extent of the surgical procedure performed.

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Meningiomas of the ventral foramen magnum

Neurosurgical FOCUS ◽

10.3171/foc.1999.6.6.8 ◽

1999 ◽

Vol 6 (6) ◽

pp. E7 ◽

Cited By ~ 2

Author(s):

Kenan I. Arnautovic ◽

Ossama Al-Mefty ◽

Muhamed Husain

Keyword(s):

Cranial Nerve ◽

Nerve Palsy ◽

Foramen Magnum ◽

Karnofsky Performance Scale ◽

Recurrent Tumor ◽

Lower Cranial Nerve ◽

Cranial Nerve Palsies ◽

Favorable Influence ◽

Prolonged Hospital

Meningiomas of the ventral foramen magnum are rare lesions that account for less than 3% of all meningiomas. Their treatment remains one of the most challenging among all meningiomas. The authors comprehensively analyzed multiple features in the series of patients who harbored these lesions. The authors conducted a retrospective study of 18 patients who harbored lesions in the ventral foramen magnum (mean follow up 40 months) in whom surgery was performed via a transcondylar approach. Sixteen patients underwent surgery for the first time: in 12 patients (75%) gross total, in two (12.5%) near total, and in two (12.5%) subtotal tumor removal was achieved. The remaining two patients were treated for a recurrent tumor. Karnofsky Performance Scale (KPS) scores obtained at follow-up review demonstrated statistically significant improvement compared with those obtained preoperatively. The extent of surgical resection and preoperative KPS score were variables that demonstrated statistically significant favorable influence on outcomes. Ninth and 10th cranial nerve palsies were the most common complication that contributed to the prolonged hospital stay; six patients who experienced nerve palsy preoperatively worsened postoperatively, and four other patients developed nerve palsy after surgery. There were no perioperative deaths. Four patients died during the follow-up period. One patient died of multiple myeloma; another, who underwent surgery for a recurrent tumor, died 3 years after this second surgery from new tumor recurrence at 80 years of age; and the remaining two patients died 1.5 and 5 months postsurgery of pulmonary embolus and endocarditis, respectively. Meningioma of the ventral foramen magnum can be radically removed in a majority of patients in whom complications will be frequent but transient, resulting from lower cranial nerve palsies. The radical removal of a recurrent tumor provides for the patient a relatively long, stable follow-up period. Patients presenting with a low KPS score have a poor prognosis. Early diagnosis and treatment are recommended.

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