Lacrimal gland pleomorphic adenoma: a narrative review / Adenoma pleomórfico de glândula lacrimal: uma revisão narrativa

ABSTRACT We present a literature review of 57 publications describing this pathology, published from the year 2012. In all these studies patients were reported to depict a slow-growing, motionless mass, which is painless at most times. All cases were managed by total excision, except for one report where adjuvant radiotherapy was applied. Among the several therapeutic strategies, the total tumor resection, preserving the tumor pseudocapsule intact, appears to be a consensus in treating the disease efficiently. Furthermore, fine-needle aspiration biopsy, including the assessment of genetic alterations, has proved to be a valuable tool in the diagnosis of challenging cases. Our literature survey also suggests that an incisional biopsy before the surgery may lead to the pseudocapsule disruption, thus considerably increasing the chances of adenoma recurrence, enabling its malignization. At present, genetics studies indicate that the molecular aberrations involved in the adenoma are similar to those represented in the salivary gland tumor pathogenesis. Further, in the recurrent cases, the pathology becomes difficult to treat and multiple surgeries may be required, occasionally, leading to radical surgery treatment.

RESUMO Uma revisão narrativa da literatura de 57 publicações que descrevem esta patologia, publicada a partir de 2012. Os pacientes têm uma massa de crescimento lento e imóvel, que na maioria das vezes é indolor. Todos os casos foram tratados por excisão total, com exceção de um relatório de radioterapia adjuvante. Entre as estratégias terapêuticas encontradas, a ressecção total do tumor, preservando a pseudocápsula tumoral intacta, parece ser um consenso. Alternativamente, a biópsia por aspiração de agulha fina incluindo a avaliação de alterações genéticas pode representar uma ferramenta valiosa nos casos diagnósticos desafiadores. Uma biópsia incisional antes da cirurgia não é recomendada, pois a ruptura da pseudocápsula aumenta consideravelmente a recorrência do adenoma, permitindo até mesmo sua malignização. Com relação à genética, estudos atuais indicam que as aberrações moleculares envolvidas no adenoma são semelhantes às da patogênese do tumor da glândula salivar. Para casos de recorrência, a patologia torna-se difícil de tratar e múltiplas cirurgias podem ser necessárias, às vezes levando a um tratamento cirúrgico radical.

Clinical behavior of recurrent pleomorphic adenoma in the palate: a systematic review

Purpose: This systematic review analyzed the clinical behavior and odds of malignancy of the palatal recurrent pleomorphic adenomas. Methods: Systematic review of patients with recurrent pleomorphic adenoma arising in the palate. Database search: MEDLINE, Scopus, Web of Science, Cochrane, EMBASE, Virtual Health Library, Google Scholar, and OpenGrey. A binomial logistic regression was performed to assess the odds of detecting recurrence five, 10 and 20 years after the treatment of primary tumor. Results: Thirteen studies (n = 18 patients) out of 336 were included. The recurrent pleomorphic adenoma in palate was more common in females (61.6%), average age was 49 years old (range 9-73 years old). Four patients progressed to malignant transformation. The odds ratio (OR) of detecting a recurrence at 10 (OR = 5.57; 95% confidence interval - 95%CI 1.13-27.52), and 20 years (OR = 18.78; 95%CI 3.18-110.84) after treatment of primary pleomorphic adenoma was significantly higher than at one-year follow-up. Conclusions: The recurrence of pleomorphic adenoma in palate remains a rare event of late occurrence. It mainly affects middle-aged female and carries a risk of malignant transformation. Although uncommon, patients with palatal pleomorphic adenoma should be warned about the possibility of recurrence or malignant transformation of tumor at advanced ages.

EZH2 immunoexpression in pleomorphic adenoma and adenoid cystic carcinoma and clinicopathological features

Abstract The aim of this study was to evaluate the expression of the EZH2 protein and describe the clinical and microscopic characteristics of adenoid cystic carcinoma (ACC) and pleomorphic adenoma (PA). The study included 16 ACC cases and 12 PA. All ACC and PA cases were positive for EZH2 and the ACC samples showed significantly higher EZH2 expression. The clinical and microscopic covariates were described in relation to EZH2 staining in ACC samples. The highest mean values of EZH2 were observed in cases with local metastasis, recurrence, perineural invasion, and predominantly cribriform growth pattern without solid areas. EZH2 is a potential marker of malignancy.

Adenoma Hepatocelular. A propósito de un caso

El Adenoma Hepatocelular (AH) es un tumor hepático benigno, su diagnóstico ha avanzado gracias a los avances en los métodos moleculares, facilitaron dividirlos en subtipos, con diferentes pronósticos e indicaciones terapéuticas. Se presenta el caso clínico de una paciente, de 40 años con hallazgo ecográfico de tumor hepático, la Tomografía de Abdomen y Pelvis voluminosa lesión sólida heterogénea, en la Resonancia Magnética compatible con Adenoma esteatósico (asociado a mutación HNF1 alfa). Se decide tratamiento quirúrgico, con resección de los segmentos 6 y 7. La Anatomía patológica concluye: Compatible con el subtipo inflamatorio. Los Adenoma hepáticos (AH) son tumores raros, solitarios de estirpe epitelial, benignos. Se presentan en mujeres de edad fértil y asociado al consumo de anticonceptivos orales y estrógenos. Estos tumores predominan en hígado derecho, con proliferación de células parecidas a los hepatocitos normales, pero desorganizados y sin arquitectura lobular normal, sin ductos biliares ni tejido conectivo de sostén. Los AH así como el resto de los tumores hepáticos benignos, han aumentado su incidencia de la mano con el avance de la imagenología abdominal. La importancia de la diferenciación con el resto de los tumores hepáticos benignos surge del potencial maligno de éstos. Podemos clasificar a los pacientes según el perfil molecular asociado a marcadores inmunohistoquímicos. Los estudios de imagen son fundamentales para la diferenciación tumoral en diagnóstico y planear la terapéutica. El tratamiento será individualizado, determinada por la clínica, la variedad de subtipos, y la evolución. Debido a la complejidad de la enfermedad, el tratamiento de la HA es uno de los mejores ejemplos de abordaje individualizado en unidades hepatobiliares.

Hepatocellular adenoma (HA) is a benign liver tumor, its diagnosis has advanced thanks to advances in molecular methods, which facilitated its division into subtypes, with different prognoses and therapeutic indications. We present the clinical case of a 40-year-old patient with an ultrasound finding of a liver tumor, a voluminous heterogeneous solid lesion on a CT scan of the abdomen and pelvis, compatible with a steatotic adenoma on MRI (associated with HNF1 alpha mutation). Surgical treatment was decided, with resection of segments 6 and 7. The pathology concluded in short: Compatible with the inflammatory subtype. Hepatic adenomas (HA) are rare, solitary, benign epithelial tumors. They occur in women of childbearing age and associated with the consumption of oral contraceptives and estrogens. These tumors predominate in the right liver, with proliferation of cells similar to normal hepatocytes, but disorganized and without normal lobular architecture, without bile ducts or supporting connective tissue. HA, as well as the rest of the benign liver tumors, have increased their incidence in the hand with the advancement of abdominal imaging. The importance of differentiation with the rest of the benign liver tumors arises from the malignant potential of these. We can classify patients according to the molecular profile associated with immunohistochemical markers. Imaging studies are fundamental for tumor differentiation in diagnosis and therapeutic planning. The treatment will be individualized, determined by the clinic, the variety of subtypes, and the evolution. Due to the complexity of the disease, the treatment of AH is one of the best examples of an individualized approach in hepatobiliary units.

O adenoma hepatocelular (AH) éum tumor benigno do fígado, seu diagnóstico avançougraçasaosavanços dos métodos moleculares, que facilitaramsuadivisão em subtipos, com diferentes prognósticos e indicaçõesterapêuticas. Apresentamos o caso clínico de umadoente de 40 anoscomachadoultrassonográfico de tumor hepático, volumosalesão sólida heterogénea à TC de abdómen e pelve, compatívelcom adenoma esteatótico à RM (associado a mutação HNF1 alfa ). Optou-se por tratamentocirúrgico, comressecção dos segmentos 6 e 7. A patologiaconcluiu-se resumidamente: Compatívelcom o subtipo inflamatório. Os adenomas hepáticos (AH) são tumores epiteliais raros, solitários e benignos. Ocorrem em mulheres em idadereprodutiva e associadasao consumo de anticoncepcionaisorais e estrogênios. Esses tumores predominam no fígadodireito, comproliferação de células semelhantesaoshepatócitosnormais, porém desorganizados e semarquitetura lobular normal, sem ductos biliares outecido conjuntivo de sustentação. O HA, assim como os demais tumores hepáticos benignos, têm aumentado suaincidêncianamãocom o avanço da imagem abdominal. A importância da diferenciaçãocom os demais tumores hepáticos benignos decorre do potencial maligno destes. Podemos classificar os pacientes de acordocom o perfil molecular associado a marcadores imuno-histoquímicos. Os estudos de imagemsãofundamentais para a diferenciação tumoral no diagnóstico e planejamentoterapêutico. O tratamento será individualizado, determinado pela clínica, variedade de subtipos e evolução. Pela complexidade da doença, o tratamento da HA é um dos melhoresexemplos de abordagem individualizada nas unidades hepatobiliares.

Changes of macular retinal structure and microcirculation in patients with pituitary adenoma observed by optical coherence tomography and optical coherence tomography angiography / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To observe the changes of macular retinal structure and microcirculation in patients with pituitary adenoma(PA)by optical coherence tomography(OCT)and optical coherence tomography angiography(OCTA).METHODS: Cross-sectional study. A total of 40 PA patients treated at the department of neurosurgery, Affiliated Hospital of Guangdong Medical University from September 2021 to March 2023 were included as PA group, and 42 age- and gender-matched healthy volunteers were selected as normal control group. All patients underwent visual field, OCT and OCTA examinations, and the correlation of ocular parameters in PA patients was analyzed.RESULTS:The vessel density(VD)of each retinal layer in the macular area of the PA group was lower than that of the normal control group, and the superficial vascular complex(SVC)-VD in the macular area was positively correlated with the thickness of the macular ganglion cell complex(mGCC)(except the nasal side of the inner ring and the lower part of the outer ring; P<0.05). The thickness of mGCC in each quadrant of the macular area and the thickness of the circumpapillary retinal nerve fiber layer(CP-RNFL)in each quadrant were negatively correlated with the mean defect(MD)value of the visual field(P<0.05), and the area of the foveal avascular zone(FAZ)was positively correlated with the MD value(P<0.05).CONCLUSION:The combination of OCT and OCTA can fully understand the microscopic changes of retinal structure and microcirculation function in PA patients, which is of great value in evaluating the preoperative visual function of PA patients.

Hypercalcemic crisis as a presentation of primary hyperparathyroidism / Crisis hipercalcémica como presentación de hiperpara-tiroidismo primario

Abstract Hyperparathyroidism-induced hypercalcemic crisis (HIHC) is an unusual state of marked progressive pri mary hyperparathyroidism (PHPT). Patients have severe hypercalcemia and may have severe symptoms such as kidney failure, acute pancreatitis, and mental changes. PHPT is due to the presence of a single gland adenoma/ disease in 80 to 85%; parathyroid carcinoma is reported in <1%. Among patients with adenoma, atypical parathy roid tumor can be found infrequently. Parathyroidectomy is the only curative approach for PHPT. In this report we present three cases of HIHC due to giant parathyroid adenomas (GPAs), one of them with histopathological characteristics of an atypical parathyroid tumor, with satisfactory evolution after parathyroidectomy.

Resumen La crisis hipercalcémica inducida por hiperparatiroi dismo (HIHC) es un estado inusual de hiperparatiroidis mo primario progresivo y marcado (HPTP). Los pacientes tienen hipercalcemia grave y pueden tener síntomas graves como insuficiencia renal, pancreatitis aguda y cambios mentales. El HPTP se debe a la presencia de un adenoma/enfermedad de una sola glándula en 80 a 85%; el carcinoma de paratiroides se informa en <1%. Entre los pacientes con adenoma, el tumor paratiroideo atípico se puede encontrar con baja frecuencia. La paratiroidec tomía es el único abordaje curativo del HPTP. En este reporte presentamos tres casos de HIHC por adenomas paratiroideos gigantes (APGs), uno de ellos con características histopatológicas de tumor paratiroideo atípico, con evolución satisfactoria luego de paratiroidectomía.

A unique coexistence of a plurihormonal pituitary adenoma with granulomatous hypophysitis

Dual pathology in the pituitary gland is a unique phenomenon. Coexistence of a pituitary adenoma with primary hypophysitis has been reported rarely with very few cases in the literature. Among the primary hypophysitis, primary granulomatous subtype has been proposed to be idiopathic and autoimmune in nature. Plurihormonal pituitary adenomas produce hormones of more than one different pituitary cell lineage. Pituitary adenoma with a single hormonal content has been documented with concurrent primary granulomatous hypophysitis. The present case describes the unique coexistence of a plurihormonal adenoma showing somatotroph, lactotroph, and corticotroph lineage with primary granulomatous inflammation in the sellar region in a 36-year-old woman.

Pancreatitis aguda en el escenario de hipercalcemia por hiperparatiroidismo primario / Acute pancreatitis in the setting of hypercalcemia due to primary hyperparathyroidism

Introducción: describir el caso de un paciente con pancreatitis aguda secundaria a hipercalcemia por hiperparatiroidismo prImario. Esta es una causa poco frecuente de pancreatitis, asociada a morbimortalidad significativa en caso de no ser diagnosticada oportunamente Caso clínico: un hombre de 44 años, con antecedente de pancreatitis de presunto origen biliar que había requerido previamente colecistectomía, consultó por dolor abdominal y náuseas. Los estudios complementarios fueron compatibles con un nuevo episodio de pancreatitis aguda. Presentaba hipercalcemia y hormona paratiroidea (PTH) elevada, configurando hiperparatiroidismo primario. La gammagrafía informó hallazgos compatibles con adenoma paratiroideo. Se inició tratamiento con reanimación hídrica y analgesia con adecuada disminución de calcio sérico y resolución de dolor abdominal. Después de la paratiroidectomía se logró normalizar los niveles de calcio y PTH. Discusión: la pancreatitis aguda es una condición potencialmente fatal, por lo que la sospecha de causas poco frecuentes como la hipercalcemia debe tenerse en cuenta. El tratamiento de la hipercalcemia por adenoma paratiroideo se basa en reanimación hídrica adecuada y manejo quirúrgico del adenoma, con el fin de evitar recurrencia de pancreatitis y mortalidad. (AU)

Introduction: we describe the case of a patient with acute pancreatitis secondary to hypercalcemia due to primary hyperparathyroidism. This is a rare cause of pancreatitis associated with significant morbidity and mortality if not diagnosed in time. Clinical case: a 44-year-old man with a history of pancreatitis of presumed biliary origin, which had previously required cholecystectomy, consulted for abdominal pain and nausea. The laboratory findings were compatible with a new episode of acute pancreatitis. He presented hypercalcemia and an elevated parathyroid hormone (PTH), configuring primary hyperparathyroidism. Scintigraphy was performed, yielding findings compatible with parathyroid adenoma. Treatment with fluid resuscitation and analgesia was started, resulting in an adequate decrease in serum calcium and resolution of abdominal pain. After parathyroidectomy, calcium and PTH levels were normalized. Discussion: acute pancreatitis is a potentially fatal condition; therefore the suspicion of rare causes, such as hypercalcemia, should be considered. The treatment of hypercalcemia due to parathyroid adenoma is based on adequate fluid resuscitation and surgical management of the adenoma, to avoid recurrence of pancreatitis and death. (AU)

Hipercalcemia asociada a hiperparatiroidismo primario: enfoque y manejo a partir de un caso clínico

Introducción: el calcio es el electrolito más abundante del cuerpo humano y la hipercalcemia es el trastorno común causado normalmente por el hiperparatiroidismo primario o malignidad, su manejo depende de la presentación y causa subyacente. Además, una proporción de casos se presentan como una emergencia, lo que conlleva a una mortalidad significativa. Objetivo: mostrar un caso inusual de presentación clínica de hipercalcemia asociada a hiperparatiroidismo primario y, asimismo, dar una breve revisión acerca del enfoque y el manejo de esta patología. Presentación del caso: paciente femenina de 32 años, antecedente de adenoma paratiroideo no resecado y pancreatitis, asiste por tres días de dolor abdominal de tipo cólico y de moderada intensidad, acompañado de episodios eméticos de contenido alimentario, paraclínicos iniciales con hipercalcemia severa, electrocardiograma con bloqueo auriculoventricular grado I, gases arteriales con alcalosis respiratoria aguda e hiperlactatemia. También se le practicó un TAC de abdomen donde este apareció con tumores pardos. Se ingresó a la UCI para la administración de líquidos endovenosos, diuréticos de asa y cinacalcet, pero no presentó mejoría, por lo que se indicó terapia de hemodiálisis. Discusión y conclusión: la hipercalcemia es un hallazgo frecuente. El hiperparatiroidismo primario y la neoplasia maligna son las dos causas más frecuentes de aumento de los niveles de calcio sérico y, en conjunto, representan alrededor del 90 % de todos los casos, donde los valores en suero varían entre el calcio total (8,5 y 10,5 mg/dl) y el iónico (1,16-1,31). La concentración sérica de Ca 2+ está estrechamente relacionada por las acciones de la hormona paratiroidea y el calcitriol, donde el hiperparatiroidismo primario ocurre como resultado de adenomas, hiperplasias y carcinoma. Las manifestaciones clínicas y la severidad van a estar correlacionadas con el tiempo de duración de la enfermedad, los niveles de calcio y de PTH. Dentro del tratamiento, este será guiado por su causa, sin embargo, es posible clasificarlo en tratamiento urgente y no urgente. Además, el enfoque de la hipercalcemia aguda severa es un reto diagnóstico dadas las múltiples causas que pueden llevar a este trastorno hidroelectrolítico y la rápida instauración de tratamiento que se requiere cuando es detectada.

Background: Calcium is the most abundant electrolyte in the human body, hypercalcemia is a common disorder usually caused by primary hyperparathyroidism or malignancy. A proportion of cases presenting as an emergency, leading to significant mortality. The management of hypercalcemia depends on the presentation and underlying cause. Purpose: to present an unusual case of clinical presentation of hypercalcemia associated with primary hyperparathyroidism, as well as to give a brief review about the approach and management of this pathology. Case presentation: A 32-year-old female patient, with a history of unresected parathyroid adenoma and pancreatitis, attended for 3 days of abdominal pain, moderate intensity, accompanied by emetic episodes of food content, initial paraclinical findings showed severe hypercalcemia, electrocardiogram with block grade I atrioventricular, arterial gases with acute respiratory alkalosis and hyperlactatemia. CT of the abdomen with brown tumors. She was admitted to the ICU for administration of intravenous fluids, loop diuretics, and cinacalcet without showing any improvement, so hemodialysis therapy was indicated. Discussion and conclusion: hypercalcemia is a frequent finding. Primary hyperparathyroidism and malignancy are the two most common causes of elevated serum calcium levels, together accounting for about 90 % of all cases. Serum values vary between total calcium 8.5 and 10.5 mg/dl and ionic 1.16- 1.31. Serum Ca 2+ concentration is closely related by the actions of parathyroid hormone and calcitriol. Primary hyperparathyroidism occurs as a result of adenomas, hyperplasias, or carcinoma. The clinical manifestations and severity will be correlated with the duration of the disease, calcium and PTH levels. Within the treatment, this will be guided by its cause, however, it is possible to classify it into urgent and non-urgent treatment. The approach to severe acute hypercalcemia is a diagnostic challenge given the multiple causes that can lead to this hydroelectrolyte disorder and the rapid establishment of treatment that is required when it is detected.

Pleomorphic Adenoma of the Hard Palate: A Multidisciplinary Approach

Pleomorphic adenoma is the most common salivary gland tumor accounting for 80% of all major salivary gland tumors. It is a benign salivary gland neoplasm that constitutes 3% to 10% of the neoplasms in the head and neck region.1 Salivary gland neoplasms represents less than 1% of all tumors. This article is being showcased as a special case due to the fact it was done at a Taluk Hospital and also because ENT and oromaxillofacial surgeons were involved during the surgery.

McKittrick Wheelock syndrome secondary to adenocarcinoma of the rectum: case report and diagnostic considerations

Introduction: McKittrick-Wheelock syndrome is a rare entity characterized by chronic diarrhea, acute kidney injury, and hydroelectrolytic imbalance associated with a large rectal tumor, frequently a villous adenoma. Case report: A 69-year-old male with chronic diarrhea with mucus. He underwent a colonoscopy with biopsies, reporting adenocarcinoma of the rectum in situ, and underwent a robot assisted intersphincteric resection with colo-anal anastomosis and a protecitive ileostomy. Discussion: Described in 1954, this syndrome is manifested by electrolyte imbalance and acute renal injury secondary to diarrhea associated with a rectal villous adenoma, often with long lasting symptoms. The most frequent symptom being watery diarrhea with mucus. The definitive treatment consists of surgical resection. Conclusion: Although this is a rare pathology, it should be considered as a differential diagnosis in cases of chronic diarrhea associated with water and electrolyte disorders. (AU)

Clinical and radiologic outcomes of pleomorphic adenoma and adenoid cystic carcinoma of the lacrimal gland / Desfechos clínicos e radiológicos do adenoma pleomórfico e do carcinoma adenoide cístico da glândula lacrimal

ABSTRACT Purpose: To compare the radiologic and clinical features of primary lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma. Methods: This study retrospectively reviewed imaging findings and medical records of lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma. Results: Eleven patients with pleomorphic adenoma and 16 patients with adenoid cystic carcinoma were evaluated. There were no statistically significant differences between groups regarding age or sex. Proptosis was the most common presenting symptom in both groups. Adenoid cystic carcinomas were more likely to present with a palpable mass, diplopia, pain, and sensory loss than pleomorphic adenomas, although the differences were not statistically significant between groups. Furthermore, there were no significant differences in terms of homogeneity and globe indentation between lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma on computed tomography (CT)(all p>0.05). The rates of bone invasion, tumor calcification, and wedge sign were significantly higher in adenoid cystic carcinomas, and bone remodeling was statistically significantly higher in pleomorphic adenomas, on CT(all p<0.05). Pleomorphic adenomas were significantly more likely to show well-defined margins, lobulated contours, heterogeneous contrast enhancement, and hyperintensity on T2-weighted magnetic resonance images (all p<0.05). Conclusion: When differentiating between lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma, evaluation of radiologic features along with clinical features is of great importance. Lobulated contours may be a significant distinguishing radiologic feature suggesting pleomorphic adenoma.

RESUMO Objetivo: Comparar as características radiológicas e clínicas do adenoma pleomórfico primário e do carcinoma adenoide cístico da glândula lacrimal. Métodos: Este estudo revisou retrospectivamente os achados de imagem e os prontuários médicos de casos de adenoma pleomórfico e carcinoma adenoide cístico da glândula lacrimal. Resultados: Foram avaliados 11 pacientes com adenoma pleomórfico e 16 pacientes com carcinoma adenoide cístico. Não houve diferenças estatisticamente significativas em relação à idade e sexo. Proptose foi o sintoma de apresentação mais comum em ambos os grupos. Os carcinomas adenoides císticos foram mais propensos que os adenomas pleomórficos a apresentarem massas palpáveis, diplopia, dor e perda sensorial, mas essa diferença entre os grupos não foi estatisticamente significativa. Não houve diferenças estatísticas em termos de homogeneidade e indentação do globo ocular entre os dois tipos de tumores em imagens de tomografia computadorizada (p>0,05). Também à tomografia computadorizada, a invasão óssea, a calcificação do tumor e o sinal em cunha foram mais frequentes nos carcinomas adenoides císticos, enquanto a remodelação óssea foi mais frequente nos adenomas pleomórficos, com significância estatística para todas essas manifestações (p<0,05). À ressonância magnética, os adenomas pleomórficos foram significativamente mais propensos a terem margens bem definidas, contornos lobulados, realce heterogêneo pelo contraste e hiperintensidade na ressonância magnética ponderada em T2 (p<0,05). Conclusão: Ao se diferenciar o adenoma pleomórfico e o carcinoma adenoide cístico da glândula lacrimal, é muito importante avaliar as características radiológicas juntamente com as características clínicas. Os contornos lobulados podem ser uma característica radiológica significativamente distinta em favor do adenoma pleomórfico.

Incidentalomas o no incidentalomas: ¿cuál es la relevancia de los adenomas hipofisarios en el adulto? / Incidentalomas or Non-Incidentalomas: What is the Relevance of Pituitary Adenomas in the Adult?

Introducción: desde el punto de vista anatómico, los adenomas hipofisarios (AH) se observan en el 10% de la población. Son en su mayoría pequeños y no funcionantes. La mayoría de los incidentalomas descubiertos en estudios de imágenes con alta resolución pedidos en situaciones clínicas frecuentes, como el traumatismo craneoencefálico, el accidente cerebrovascular y las demencias, corresponden a AH indolentes. Nos preguntamos cuál es la relevancia clínica de los adenomas hipofisarios. Desarrollo: los AH clínicamente relevantes son tumores en su mayoría benignos que conllevan, en diferentes proporciones, aumento en la morbilidad y/o mortalidad de los pacientes por mecanismos relacionados con la hipersecreción hormonal, la insuficiencia hormonal y/o los efectos de masa ocupante. La prevalencia de los AH clínicamente relevantes es mayor de la que se suponía hace 20 años. Afecta aproximadamente a 1/1000 habitantes. Los más prevalentes son los prolactinomas y los adenomas no funcionantes. La acromegalia, la enfermedad de Cushing y los tumores agresivos se traducen en pacientes complejos con mayor morbimortalidad. El diagnóstico temprano y el tratamiento multimodal proveen una razonable mejoría de la sobrevida. El estudio epidemiológico de los AH clínicamente relevantes es importante para la estimación del impacto en los sistemas de salud. Conclusiones: los estudios por imágenes de mejor resolución continuarán señalando incidentalomas hipofisarios. Una evaluación cuidadosa de los pacientes podrá identificar aquellos AH clínicamente relevantes. (AU)

Introduction: from the anatomical point of view, pituitary adenomas (HA) are observed in 10% of the population. They are mostly small and non-functioning. Most incidentalomas discovered in high-resolution imaging studies ordered in frequent clinical situations, such as head trauma, stroke and dementia, correspond to indolent HA. We wonder what is the clinical relevance of pituitary adenomas. Development: clinically relevant HAs are mostly benign tumors that lead, in different degrees, to an increased morbidity and/or mortality in patients by mechanisms related to hormone hypersecretion, hormone insufficiency and/or occupying mass effects. The prevalence of clinically relevant HA is higher from what was assumed 20 years ago. It affects approximately 1/1000 of the population. The most prevalent are prolactinomas and non-functioning adenomas. Acromegaly, Cushing's disease and aggressive tumors make for complex patients with increased morbidity and mortality. Early diagnosis and multimodal treatment provide a reasonable improvement in survival. Epidemiological study of clinically relevant HAs is important for estimating the impact on health systems. Conclusions: Higher-resolution imaging studies will continue to highlight pituitary incidentalomas. Careful evaluation of patients will identify clinically relevant HAs. (AU)

OCULAR FINDINGS OF TUBEROUS SCLEROSIS – A CASE REPORT

The term “Neurocutaneous syndromes” also known as Phakomatoses refers to a broadcategory of congenital diseases that includes anomalies of neuroectodermal and occasionallymesodermal development, hence commonly involving the skin, eyes and central nervoussystem. The term Phacomatoses was coined by Van Der Hoeve in 1921. Tuberous sclerosisis one type of Neurocutaneous syndromes. The word "tuberous sclerosis" comes from thedistinctive cortical tubers that Désiré-Magloire first described. Tuberous sclerosis is ahereditary disorder that affects numerous body systems. A 30 year old female diagnosed asTuberous sclerosis was referred by Dermatology Department. Detailed ophthalmicexamination was done. Patient was diagnosed with Adenoma sebaceum of upper eyelids inboth eyes and multiple retinal hamartoma in left eye.

Application of the Milan System for Reporting Salivary Gland Cytology and Risk Stratification by Cytohistological Correlation

Introduction:Salivary gland neoplasms are uncommon tumors comprising <3%–10% of all head‑and‑neck neoplasms. Recent WHO classification of salivary gland tumor added many newer entities; however, no definite risk‑stratification system is specified to predict the likelihood of malignancy for each diagnostic category. The present study is designed to evaluate the salivary gland aspirates using the Milan System for Reporting Salivary Gland Cytology for reporting correlating with histological diagnosis which will lead to determine the malignancy risk for each diagnostic category. Risk of malignancy is calculated by the ratio of cytopathology cases with a malignant histopathology to the total number of cytopathology cases with follow‑up histopathology for that particular category. MaterialsandMethods: The present study was a cross‑sectional study done prospectively over a period of 2 years, and a total of 72 participants were selected. This study characterized the cytological features of spectrum of salivary gland lesions varying from benign to malignant. The lesions were evaluated and classified cytologically according to “the Milan System for Reporting Salivary Gland Cytopathology” for reporting salivary gland neoplasms. Histopathological correlation was done with cytological diagnosis wherever possible. Results: In our study of 72 patients with salivary gland lesions with the mean age of the patient being 50.6 years and maximum number of lesions involved the parotid gland followed by the submandibular gland and sublingual gland. Majority of the lesions in the present study were nonneoplastic in nature followed by lesions benign in nature. Malignant lesions were least common in occurrence. Among 72 cases, majority of the cases were seen in Milan category II (nonneoplastic) consisting of 34 cases (47.2%), followed by 31 cases (43.1%) in Milan category IV (benign). Final diagnostic categorization of 72 cases of salivary gland lesions was done according to the Milan system and the histopathological correlation was available in 23 of these cases. Out of the 34 cases in Milan system category II (nonneoplastic), histopathological evaluation was done in 2 cases. Both the cases were benign in nature, 31 cases in Milan system category IV (benign) histopathological evaluation was done in 16 cases where 15 cases were benign in nature and 1 lesion was malignant. Four cases were put in category VI of the Milan system, histopathological evaluation was done in all the 4 cases which were all malignant in nature. Conclusion: The current study validates fine‑needle aspiration cytology as a cost‑effective and noninvasive procedure for differentiating between benign and malignant lesions of the salivary gland, information of critical importance when determining the patient’s next course of treatment.

Analysis of Various Histopathological Patterns in Pleomorphic Adenoma - An Institutional Case Study

Introduction: Pleomorphic adenoma, the most common benign mixed salivary gland tumor which accounts for 70-80%, is a biphasic tumor with dual origin from epithelial and myoepithelial elements. It shows a malignant transformation rate of 6%. Microscopically, pleomorphic adenoma characteristically displays vast morphologic diversity. This present study is done to analyze prevalence rates and histomorphological variations to better elucidate the pattern of occurrence, diagnosis and treat- ment plan of pleomorphic adenoma. Materials and Methods: This are a retrospective study which was conducted in a private institution, Chennai. Based on the proportion of parenchymal and stromal tumor components, the cases were classified into four subtypes as proposed by Foote and Frazell. Morphological patterns, cellular patterns, capsular alterations and Stromal components were analyzed and statisti- cally evaluated. Results: Our analysis showed an overall 0.87% of pleomorphic adenoma cases being reported were associated with minor salivary glands followed buccal mucosa and palate. All the reported cases showed Type II pattern with histological cellular pat- tern of spindle cells and ductal morphological pattern. Conclusion: We observed the great diversity of morphological aspects of the stroma in pleomorphic adenoma, in which many cases showed variation in morphology and cellularity associated with tumor location.

Basal Cell Adenocarcinoma - 2 Case Series of a Rare Entity

Introduction: Basal cell adenocarcinoma is a rare well recognized low grade malignant salivary gland tumor often resembles basal cell adenoma. the infiltrating growth pattern and likelihood of vascular and perineural involvement distinguishes basal cell adenocarcinoma with basal cell adenoma. Case presentation: Here we present histopathological and immune histochemical analysis of two such rare cases of basal cell adenocarcinoma and review of literature is discussed. Conclusion: Basal cell adeno carcinoma is a low grade malignancy and doesn’t metastasize. With only few hundreds of cases that has been reported in literature, basal cell adeno carcinoma should be included as differential diagnosis in salivary gland tumors in order not to miss the diagnosis

Cytological spectrum of diseases in goiter in Kadapa, a semiurban city in southern part of India – A 2-year retrospective study

Background: Thyroid gland produces thyroid hormone, thyroxine. Goiter is an indefinite term applied to the enlargement of thyroid gland. The normal thyroid gland in non-goitrous state weighs 25–35 g. Thyroid gland disorders are common endocrine disorders in the world. Aims and Objectives: This is a retrospective study done to know the age and gender distribution of goiter and various pathological conditions in goiter. This study showed that the incidence is common in 3rd–5th decade of life and nodular and colloid goiter are the common causes of goiter followed by follicular adenoma and papillary carcinoma. Materials and Methods: The data were collected from cytology registers of Pathology Department of FIMS Hospital, Image Diagnostics and Seetha Diagnostics, Kadapa, during the 2-year period from January 2021 to December 2022. The data collected as above were systematically analyzed and reviewed for age and sex distribution, clinical presentation, and cytological diagnosis. Results: Majority of the patients were female accounting for 324 cases (94.18%) and 20 patients (5.81%) were male. The maximum incidence is in young females between 31 and 40 years age group (24.70%) followed by 41–50 years age group (23.83%) and then in 21–30 years age group (22.38%). All together, the overall maximum incidence is seen in females between 21 and 50 years age group (70.91%). The most common pathological disease is nodular goiter followed by colloid nodule, both being iodine deficiency goiters. Conclusion: The study showed the thyroid disorders are more common in females. The non-neoplastic lesions constitute the predominant cause for goiter followed by benign neoplasms and then malignant neoplasms.

Metabolic syndrome and Visceral Adiposity Index in non-functional adrenal adenomas

ABSTRACT Objective: We aimed to identify metabolic dysfunction in non-functioning adrenal adenomas (NFAAs) and Visceral Adiposity Index (VAI) predictability in the practical estimation of metabolic syndrome (MetS) in NFAAs. Subjects and methods: 134 NFAA patients and 68 control subjects matched for age, sex, and body mass index (BMI) were included in the study. After physical, biochemical, and endocrine evaluation, IDF and NCEP ATP III criteria were used to determine MetS. HOMA-IR and VAI were calculated for both study group subjects. Results: MetS was significantly higher in the NFAA patients. The incidence of MetS by IDF and NCEP criteria was 52.9%,48.5% in the NFAI and 32.3%,30.8% in the control group (p < 0.01, p = 0.02). The risk of MetS was increased in NFAA (75.6 vs. 24.4%, p = 0.017, OR = 1.34, 95% CI = 1.06-1.68). Glucose, HOMA IR, hypertension, and VAI were significantly increased in NFAA patients. The risk of MetS was independently associated with high VAI (79.2 vs. 20.8%, p = 0.001, OR = 2.22; 95% CI = 1.70-2.91). Conclusion: MetS, insulin resistance, and VAI are more prevalant in NFAA patients than in healthy individuals. VAI can be used with high specificity to estimate MetS in NFAA patients.

Apoplejía pituitaria e infección por SARS-COV-2 (COVID-19) / Pituitary apoplexy and SARS-CoV-2 (COVID-19) infection

Resumen La infección por coronavirus (SARS-CoV-2/COVID-19) es responsable de un diverso rango de manifestaciones extrapulmonares entre las cuales se destaca el compromiso neurológico directo o indirecto. La apoplejía hipofisaria es una entidad médica y neuroquirúrgica de gravedad variable que suele asociarse a la presencia de un adenoma subyacente. Un escaso número de reportes han vinculado estas dos enfermedades entre sí. Este trabajo describe un caso de apoplejía hipofisaria manifestada clínicamente en for ma concomitante al diagnóstico de COVID 19, en un paciente con macroadenoma hipofisario desconocido y su manejo terapéutico, revisando los mecanismos potencialmente subyacentes al nexo entre ambas entidades.

Abstract Coronavirus disease (SARS-CoV-2/COVID-19) is responsible for a wide variety of extrapulmonary manifestations, among which direct or indirect neurological compromise stands out. Pituitary apoplexy is a clinical and neurosurgical entity of variable severity, usually associated with a pituitary adenoma. Literature is scarce regarding the association between these diseases. This paper reports a case of pituitary apoplexy, manifested concomitantly to the diagnosis of COVID 19 in a patient with unknown pituitary macroadenoma, and its therapeutic management, reviewing the mechanisms potentially underlying the link between both entities.