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Αλέξανδρος Γ. Σφακιανάκης

Monday, May 24, 2021

Endoscopic, Endonasal Transsphenoidal Surgery for Tumors of the Sellar and Suprasellar Region: A Monocentric Historical Cohort Study of 369 Patients

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Front Oncol. 2021 May 7;11:643550. doi: 10.3389/fonc.2021.643550. eCollection 2021.

ABSTRACT

BACKGROUND: The endoscopic endonasal transsphenoidal approach (EETA) is an established technique for the resection of a large variety of benign sellar and suprasellar lesions, mostly pituitary adenomas. It has clear advantages over the microscopic approach, like a superior close-up view of the relevant anatomy and the tumor-gland interface, an enlarged working angle, as well as an increased panoramic vision inside the surgical area. We have been performing the EETA for over a decade, and this study will focus on perioperative and postoperative outcomes and complications and their association with the learning curve.

MATERIAL AND METHODS: All patients in our tertiary referral center (n = 369) undergoing an EETA for a lesion of the sellar and suprasellar region between January 1st 2008 and December 31st 2018 were included, an d data were retrospectively retrieved from the electronic patient records.

RESULTS: Median follow-up after surgery was 55 months. Pituitary adenomas (n = 322) were the most frequent pathology. Headache (43.4%) and loss of vision (29.3%) were the most common presenting symptoms. Median procedure duration was significantly longer during the initial 5 years (106 versus 79 minutes; p <0.0001), but incidence of peri- and postoperative CSF leaks in the early years was not significantly higher. Knosp grade >2 was associated with perioperative CSF leak (p =0.002), and perioperative CSF leak was associated with postoperative CSF leak (p <0.001). Almost all cases of meningitis were preceded by a postoperative CSF leak. In 22.4% of patients, tumor recurrence required additional therapy. Perioperative (iatrogenic) mortality was 0.8%. The overall hospital stay decreased over time from an average of 7 to 5 days, and the case load increased yearly (p =0.015).

CONCLUSION: The EETA is an excellent technique with complication rates comparable to or even lower than those in large microsurgical series in the literature. EETA has a significant learning curve affecting the procedure duration. Throughout the first 10 years following the transition from the microscopic approach to the EETA in our cohort, the caseload increased and hospital stay was reduced, while no increase in peri- and postoperative complications was observed.

PMID:34026618 | PMC:PMC8138557 | DOI:10.3389/fonc.2021.643550

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Pseudo-Cerebrospinal Fluid Leaks of the Anterior Skull Base: Algorithm for Diagnosis and Management

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J Neurol Surg B Skull Base. 2021 Jun;82(3):351-356. doi: 10.1055/s-0039-3399519. Epub 2019 Nov 8.

ABSTRACT

Objective Pseudo-cerebrospinal fluid (CSF) leaks are a rare cause of unilateral, watery rhinorrhea. We proposed a step-wise approach to evaluate these cases. Design It involves a single-center retrospective cohort study. Setting The setting is that of a tertiary academic medical center. Participants Ten patients with diagnosis of pseudo-CSF leak over a 21-year period were evaluated using our proposed algorithm that includes computed tomography, magnetic resonance imaging, nasal endoscopy, β-2 transferrin, intrathecal fluorescein, and surgical exploration of the anterior cranial base. Main Outcome Measures The occurrence of intracranial infection and resolution of the symptoms were evaluated at a mean follow-up of 94.4 months. Results Eight patients had history of skull base fracture or surgery. In all patients computed tomography, magnetic resonance imaging, and nasal endoscopy did not show signs of CSF leak. Beta-2 transferrin testing was performed in five patients, being negative in all of them. Intrathecal fluorescein was performed in seven patients, being negative in five and inconclusive in two. Surgical exploration was performed in five patients, definitively ruling out CSF leak. Six patients were treated with intranasal ipratropium, resolving the symptoms in all cases. Conclusion The presented algorithm provides a step-wise approach for patients with unilateral watery rhinorrhea, allowing to safely ruling out CSF leak.

PMID:34026412 | PMC:PMC8133806 | DOI:10.1055/s-0039-3399519

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Intravenous enhanced 3D FLAIR imaging to identify CSF leaks in spontaneous intracranial hypotension: Comparison with MR myelography

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Eur J Radiol Open. 2021 May 13;8:100352. doi: 10.1016/j.ejro.2021.100352. eCollection 2021.

ABSTRACT

PURPOSE: To evaluate the clinical utility of intravenous gadolinium-enhanced heavily T2-weighted 3D fluid-attenuated inversion recovery (HT2-FLAIR) imaging for identifying spinal cerebrospinal fluid (CSF) leaks in patients with spontaneous intracranial hypotension (SIH).

METHODS: Patients with SIH underwent MR myelography and post-contrast HT2-FLAIR imaging after an intravenous gadolinium injection. Two types of CSF leaks (epidural fluid collection and CSF leaks around the nerve root sleeve) at each vertebral level were compared between the 2 sequences. The total numbers of CSF leaks and vertebral levels involved were recorded for the whole spine. The sequence that was superior for the overall visualization of epidural and paraspinal fluid collection was then selected.

RESULTS: Nine patients with SIH were included in the present st udy. HT2-FLAIR imaging was equivalent or superior to MR myelography at each level for detecting the 2 types of CSF leaks. In the 2 types of CSF leaks, the total numbers of CSF leaks and levels involved were higher on HT2-FLAIR images than on MR myelography, while no significant difference was observed for CSF leaks around the nerve root sleeve. In all 9 patients, HT2-FLAIR imaging was superior to MR myelography for the overall visualization of epidural and paraspinal fluid collection.

CONCLUSION: Intravenous gadolinium-enhanced HT2-FLAIR imaging was superior to MR myelography for the visualization of CSF leaks in patients with SIH. This method can be useful for identifying spinal CSF leaks.

PMID:34026946 | PMC:PMC8134034 | DOI:10.1016/j.ejro.2021.100352

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Cerebrospinal Fluid Leaks of the Posterior Fossa: Patient Characteristics and Imaging Features

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J Neurol Surg B Skull Base. 2021 Jun;82(3):345-350. doi: 10.1055/s-0039-1697976. Epub 2019 Nov 6.

ABSTRACT

Objective The main purpose of this article is to investigate the prevalence and features of posterior fossa defects (PFD) in spontaneous cerebrospinal fluid leaks (sCSFL). Design This is a retrospective case series. Setting Tertiary skull base center. Participants Consecutive adults undergoing lateral skull base repair of sCSFL between 2003 and 2018. Main Outcome Measures The following data were collected: demographics, comorbidities, radiology and intraoperative findings, and surgical outcomes including complications and need for revision surgery or shunt placement. Patients with incomplete data or leaks following skull base surgery, trauma, or chronic ear disease were excluded. Results Seventy-one patients (74% female, mean age 56.39 ± 11.50 years) underwent repair of spontaneous lateral skull base leaks. Eight ears (7 patients, 11.1%) had leaks involving the posterior fossa plate in addition to defects of the tegmen mastoideum (50%), tegmen tympani (25%), or both (25%). Patients with PFDs more often had bilateral tegmen thinning on imaging (75%, odds ratio [OR]: 10.71, 95% confidence interval [CI]: 2.20-54.35, p = 0.005) and symptomatic bilateral leaks (OR: 9.67, 95% CI: 2.22-40.17, p = 0.01. All PFD patients had arachnoid granulations adjacent to ipsilateral mastoid cell opacification. However, this finding was often subtle and rarely included on the radiology report. There was no significant difference in body mass index, age, presenting complaints, or operative success between the PFD and isolated tegmen defect sCSFL cohorts. Conclusion The posterior fossa is an uncommon location for sCSFL. Careful review of preoperative imaging is often suggestive and can inform surgical approach. PFD patients are similar to those with isolated tegmen-based def ects in presentation, comorbidities, and outcomes.

PMID:34026411 | PMC:PMC8133817 | DOI:10.1055/s-0039-1697976

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Multidisciplinary Team Care in the Surgical Management of Pituitary Adenoma

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J Neurol Surg B Skull Base. 2021 Jun;82(3):295-302. doi: 10.1055/s-0039-1700498. Epub 2019 Oct 21.

ABSTRACT

Objective Despite multidisciplinary care being commonly recommended, there remains limited evidence supporting its benefits in pituitary disease management. This study aimed to assess the impact of multidisciplinary care in pituitary surgery. Methods A retrospective cohort study was performed comparing pituitary surgery outcomes among consecutive patients within a quaternary referral center in 5 years before and after introduction of a multidisciplinary team (MDT). Primary outcomes were endocrine (transient diabetes insipidus [DI], syndrome of inappropriate antidiuretic hormone [SIADH], and new hypopituitarism) and surgical (cerebrospinal fluid [CSF] leak, epistaxis, intracranial hemorrhage, and meningitis) complications, length of hospital stay, and intrasellar residual tumor. Results 279 patients (89 pre-MDT vs. 1 90 post-MDT) were assessed (age 54 ± 17 years, 48% female). Nonfunctioning adenomas were most common (54%). In the post-MDT era, more clinically functioning tumors (42 vs. 28%, p = 0.03) were treated. Transient DI and SIADH occurred less often post-MDT (20 vs. 36%, p < 0.01 and 18 vs. 39%, p < 0.01), as well as new hypothyroidism (5 vs. 15, p < 0.01). Hospital stay was shorter post-MDT (5[3] vs. 7[5] days, p < 0.001) and intrasellar residuals were less common (8 vs. 35%, p < 0.001). Complications were more frequent pre-MDT independent of tumor size, hormone status, and surgical technique (odds ratio [OR] = 2.14 [1.05-4.32], p = 0.04). Conclusion Outcomes of pituitary surgery improved after the introduction of an MDT. Pituitary MDTs may benefit both patients and the health system by improving quality of care and reducing hospital stays.

PMID:34026405 | PMC:PMC8133811 | DOI:10.1055/s-0039-1700498

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Chondroid Syringoma of the Thenar Eminence in a US Veterans Administration (VA) Patient

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Chondroid syringoma, or cutaneous mixed tumor, is an adnexal neoplasm of either apocrine or eccrine origin. It presents as a firm, dermal or subcutaneous nodule, usually between 0.5 and 3 cm. While usually solitary, cases with multiple lesions have been reported. As there is no distinctive clinical appearance, it is often misdiagnosed as another entity, such as epidermoid cyst, pilar cyst, or neurofibroma, prior to biopsy.

What you should be alert for in the history
Most patients with chondroid syringoma are in the age range of 20 to 60 years, although it has also been seen in children. The history will usually be one of a slow-growing, painless nodule. Rapid growth or ulceration should prompt concern for a malignant chondroid syringoma, although these are very rare.The malignant variant is more likely to occur on the trunk and extremities, and is more common in females.

Characteristic findings on physical examination
A chondroid syringoma is most commonly found on the head, neck, and scalp (Figure 1). Less frequently, it may also be seen on the trunk, axilla, inguinal area, and genitalia; rarely, it has been reported on the eyelid and external auditory canal. Lesions on the head and neck have a male predilection, but there is an equal sex distribution for other sites.

Figure 1.
Chondroid syringoma on the vertex of the scalp.

Expected results of diagnostic studies
Histologic examination is the definitive method to confirm the diagnosis. A well-circumscribed nodule is seen within the deep dermis or subcutis. The tumors are called "mixed" because of the biphasic pattern of epithelial structures within a mesenchymal stroma. The stroma is abundant, and can be chondroid, myxoid, fibrous, or even osseous.

The epithelial component is predominantly either apocrine or eccrine, although folliculosebaceous elements can also be seen. The apocrine type has tubular and cystic branching lumina lined by a double row of epithelial cells and demonstrating decapitation secretion. The eccrine type has ducts with smaller lumens lined by a single row of epithelial cells. The presence of cellular atypia, mitotic figures, necrosis, or an infiltrative pattern are concerning for malignancy.

The histologic appearance is similar to that of mixed tumor of the salivary gland (pleomorphic adenoma), however the latter has a much higher incidence of local recurrence and greater potential for malignant transformation.

Who is at Risk for Developing this Disease?
Most patients with chondroid syringoma are in the age range of 20 to 60 years, although it has also been seen in children.

What is the Cause of the Disease?
Although their exact origin is still unclear, chondroid syringomas have traditionally been classified into apocrine and eccrine types, based on the epithelial component of the lesion. More recently, it has been proposed that these lesions be classified as hamartomas rather than adnexal adenomas.

Systemic Implications and Complications
Chondroid syringoma is a benign growth, with little proliferative capacity. After removal, the risk of recurrence is very low. There is no systemic involvement and no associated conditions or disease states.

Most cases of malignant chondroid syringoma originate de novo, but rarely an exisiting benign mixed tumor may suddenly undergo malignant changes. The aggressive behavior of these may vary widely from local recurrence only to regional lymph node spread to bone or visceral metastases.

Treatment Options
Treatment options are sumarized in Table I.

Table I.
Surgical procedures
Excision
Electrodessication
CO2 laser
Optimal Therapeutic Approach for this Disease
Simple excision is the treatment of choice for chondroid syringoma. They are usually well-circumscribed, encapsulated, white nodules, so dissection from surrounding tissue is usually uncomplicated. Risk of recurrence is minimal, and large margins of excision are not necessary.

Other treatment options that have been used include electrodessication and CO2 laser. However, the risk of recurrence with these procedures may be slightly higher.

With their higher recurrence rate and metastatic potential, a complete surgical excision should be performed for malignant chondroid syringoma, with histologic examination of all edges to insure that no tumor remains. There are, however, no standard recommended margins for removal of these lesions.

Patient Management
No follow-up is necessary for most cases of chondroid syringoma. If the lesion is excised, it will usually not recur. For cases that have been biopsied but not excised, the patient may elect for observation instead of surgical treament. In these instances, a return visit is only necessary if any changes are noticed in the lesion.

For the rare cases of malignant chondroid syringoma, periodic follow-up is warranted after the lesion has been completely excised. The patients should be monitored for any recurrence as well as for lymphadenopathy.

Unusual Clinical Scenarios to Consider in Patient Management
The diagnosis of chondroid syringoma is not usually made clinically, and thus biopsy or excision is usually performed.

In cases of larger or deeper lesions, magnetc resonance imaging (MRI0 may be of value. Whereas there are no specific MRI features of chondroid syringoma, it can be a useful study to determine the extent and depth of the lesion as well as relation to adjacent anatomic structures.

What is the Evidence?
Yavuzer , R, Basterzi , Y, Sari , A, Bir , F, Sezer , C. "Chondroid syringoma: a diagnosis more frequent than expected". Dermatol Surg . vol. 29. 2003. pp. 179-81. (A retrospective analysis of surgeries performed for other diagnoses in which chondroid syringoma was detemined to be the actual diagnosis after postoperative histologic examination.)

Agrawal , A, Kumar , A, Sinha , A, Kumar , B, Sabira , K. "Chondroid syringoma". Singapore Med J . vol. 49. 2008. pp. 33-4. (A case report and review of the literature.)

Kaushik , V, Bhalla , R, Nicholson , C, Carpentier , J. "The chondroid syringoma: report of a case arising from the external auditory canal". Eur Arch Otorhinolaryngol . vol. 262. 2005. pp. 868-70. (A case report describing an unusual location for syringoma presentation.)

(Laxmisha , C, Thappa , D, Jayanthi , S. "Chondroid syringoma of the earlobe". J Eur Acad Dermatol Venereol . vol. 21. 2007. pp. 276-7. (A case report describing an unusual location for syringoma presentation.)

Turhan-Haktanir , N, Sahin , O, Bukulmez , A, Demir , Y. "Chondroid syringoma in a child". Pediatr Dermatol . vol. 24. 2007. pp. 505-7. (A pediatric case report.)

Awasthi , R, Harmse , D, Courtney , D, Lyons , C. "Benign mixed tumor of the skin with extensive ossification and marrow formation". J Clin Pathol. vol. 57. 2004. pp. 1329-30. (A case report describing a syringoma with unusual histologic features.)

Hafezi-Bakhtiara , S, Al-Habeeb , A, Ghazarian , D. "Benign mixed tumor of the skin, hypercellular variant". J Cutan Pathol. vol. 37. 2010. pp. 46-9. (A case report and review of the literature.)

Mandeville , J, Roh , J, Woog , J, Gonnering , R, Levin , P. "Cutaneous benign mixed tumor of the eyelid: clinical presentation and management". Ophthal Plas Recon Surg. vol. 20. 2004. pp. 110-6. (A review of diagnosis and treatment.)

Kerimoglu , U, Aydingoz , U, Ozkaya , O, Aksu , A, Ergen , F. "MRI of a benign chondroid syringoma". Brit J Radiol . vol. 79. 2006. pp. 59-61. (A description of MRI findings and review of potential uses for this imaging modality.)

Nicolaou , S, Dubec , J, Munk , P, O'Connell , J, Lee , M. "Malignant chondroid syringoma of the skin: magnetic resonance imaging features". Austral Radiol . vol. 45. 2001. pp. 240-3. (A description of MRI findings and indications for imaging these lesions.)

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Via Eplasty

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Eplasty. 2021 May 11;21:ic4. eCollection 2021.

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PMID:34025902 | PMC:PMC8110697

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Masson's Tumor of the Finger

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Intravascular papillary endothelial hyperplasia (Masson's tumor) is a benign lesion of the skin and subcutaneous tissue consisting of a reactive proliferation of endothelial cells with papillary formations related to a thrombus.

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Eplasty. 2021 May 14;21:ic5. eCollection 2021.

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PMID:34025903 | PMC:PMC8120262

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