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BackgroundMyasthenia gravis is an autoimmune neuromuscular disorder characterized by the production of abnormal autoantibodies directed against the receptors present in the neuromuscular junction. It has been the standard practice to offer thymectomy in all generalized myasthenia gravis patients despite the lack of robust evidence.ObjectivesThe objectives of this study are to describe the clinical profile and differentiate the clinical outcomes of thymectomy versus non-thymectomy and thymomatous versus non-thymomatous myasthenia gravis patients in the Philippine General Hospital.MethodologyBetween 2009 and 2014, a total of 69 postthymectomy and 16 non-thymectomy patient records were successfully retrieved. The demographic characteristics, surgical approach, and histopathologic results were obtained. The clinical outcome after 6 months or 1 year-follow-up was also determined and grouped according to the following: (1) complete remission, (2) pharmacological remission, (3) no clinical change, (4) worsening symptoms, and (5) mortality.ResultsMajority of the patients were females (68.0%) with a mean age of 39.8 years and a mean duration of myasthenic symptoms of 21 months. Using the Myasthenia Gravis Foundation of America classification, 54.1% of patients fell under Class II and 48.2% of them presented with generalized weakness. In this study, 60.8% of postthymectomy myasthenia gravis patients had either complete remission or pharmacologic remission compared with 12.5% among non-thymectomy patients (p-value <0.001). No significant difference in the clinical outcome was found between thymomatous and non-thymomatous myasthenia gravis after thymectomy (p-value = 0.29).ConclusionThis study showed that both thymomatous and non-thymomatous myasthenia gravis patients who underwent thymectomy had a higher incidence of complete stable remission and pharmacologic remission as compared with myasthenia gravis patients who did not undergo thymectomy.

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BackgroundNo previous studies have been published on poorly differentiated thyroid carcinoma (PDTC) in Southeast Asia.MethodsWe included all adult PDTC patients diagnosed using the Turin criteria at the Philippine General Hospital from 2006 to 2015. The data collected included demographics, clinical presentation, histopathology, treatment, and outcomes. Tests of association were employed to compare these data with foreign studies on PDTC, as well as with local studies on well differentiated thyroid carcinoma (WDTC) and anaplastic thyroid carcinoma (ATC).ResultsEighteen PDTC cases were identified. The median age was 62 years old, with the majority being females. All patients had goiter on presentation, and most were stage IV at the time of diagnosis. In terms of PDTC subtype, insular and trabecular patterns were equally common. Extrathyroidal extension was documented in eight patients, while five patients each had nodal and distant metastasis. All but one patient underwent surgery; however, less than half received adjuvant radioiodine therapy. The 5-year survival rate was 83%. Three patients (16.7%) died at a median of 12 months after diagnosis. Nine (50%) are still alive with persistent and/or recurrent disease at a median of 39 months after diagnosis.ConclusionThe behavior of PDTC in this Southeast Asian population was found to be similar to patterns observed in other regions, and exhibited intermediate features between WDTC and ATC. Appropriate surgery provided excellent 5-year survival rates, but the role of adjuvant therapy remains unclear. Larger studies are needed to identify prognostic factors in this population.

A 63-year old Filipino female presented with epistaxis of undisclosed duration. Examination showed a vascular, pulsating, rubbery intranasal mass involving both nasal cavities. The clinical impression was that of a nasal hemangioma. She underwent excision of the tumor and the specimen was sent for histopathologic evaluation.   The specimen consisted of several tan-brown irregular tissue fragments with an aggregate diameter of 2 cm. Microscopic examination showed a cellular spindle cell tumor underneath the respiratory mucosa. (Figure 1) The tumor cells formed a syncytial pattern arranged in whorls that were separated by thin fibrovascular bands. (Figure 2) The cells had round to oval nuclei with nuclear clearing and moderate amount of syncytial cytoplasm compatible with a meningothelial derivation. (Figure 3) There was absence of nuclear atypia, significant mitotic activity, and necrosis. Immunohistochemistry studies showed positivity for Epithelial Membrane Antigen (EMA) and Progesterone Receptors (PR), and absence of reaction for Smooth Muscle Actin (SMA) and CD34. (Figure 4) Our diagnosis was sinonasal tract meningioma.   Primary extracranial meningioma of the sinonasal cavity is rare and thus secondary extension from a primary intracranial tumor should be ruled out. It involves a wide age range with no striking gender predilection.1,2  Most common symptoms include nasal obstruction, epistaxis, exophthalmos, and a mass. Etiogenesis is not completely established and is postulated to arise from meningocytes that are entrapped during closure of midline structures, very similar to the development of meningoceles.3   Histopathologic examination discloses a spindle cell tumor arranged predominantly in whorls composed of cells showing meningothelial differentiation. Most are histologically grade 1 tumors. Grade 2 and 3 sinonasal tract meningiomas are rare.4 Histologic differential diagnoses include a glomangiopericytoma, leiomyosarcoma, and a solitary fibrous tumor/hemangiopericytoma. Close histologic evaluation with appropriate immunohistochemistry studies point to the correct diagnosis. Meningioma shows strong diffuse positivity with EMA and PR, and is usually negative for other immunohistochemistry markers such as muscle actins (for glomangiopericytoma and leiomyosarcoma), and CD34 (for solitary fibrous tumor/hemangiopericytoma).1,3  A diagnosis of primary sinonasal meningioma should not be made if an intracranial mass is identified.4   Sinonasal meningiomas are benign tumors with no documented distant metastases.1,2 Although recurrences occur in about 30% (mostly due to incomplete excision), metastasis and malignant transformation has not been reported.4             References: Gnepp, Douglas R. Diagnostic surgical pathology of the head and neck . Philadelphia : PA: Saunders/Elsevier, 2009. p. 167. Thompson LD, Gyure KA. Extracranial sinonasal tract meningiomas: a clinicopathologic study of 30 cases with a review of the literature. Am J Surg Pathol. (2000): 24(5):640-50. Ranjan G. Aiyer, V. Prashanth, Kirti Ambani, Vadish S. Bhat, Geeta B. Soni. Primary Extracranial Meningioma of Paranasal Sinuses. Indian J Otolaryngol Head Neck Surg. (2013 Aug): 65(Suppl 2): 384–387. Ro JY, Bell D, Nicolai P, Thompson LDR. Meningioma. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ. World Health Organization Classification of Head and Neck Tumours. Lyon: IARC Press. 2017. p. 50-51.