Anaplasia in childhood rhabdomyosarcoma: An under reported entity

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Anaplasia is a rare phenomenon seen in childhood RMS. The most common histologic subtype was Embryonal followed by Alveolar and spindle subtype. Design: A total of 11 cases of pediatric RMS were selected from January 2017 to June 2019 presenting at various sites. Out of 11 cases, 2 were further diagnosed as Embryonal, 2 as Alveolar, 2 as Pleomorphic, 1 as Spindle subtype and rest 4 as RMS-NOS based on morphology. All cases were positive for Desmin. The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the tumor cell (anaplastic cells) was selected as the main criterion to diagnose Anaplasia. Results: Out of the total 11 cases, anaplasia was seen in 7 cases. Out of these seven, five cases showed Focal Anaplasia (FA) (71.4%) and 2 cases showed Diffuse Anaplasia (DA) (28.6%). Out of 2 cases of Embryonal RMS one exhibited focal anaplasia (50%). One case of Spindle RMS showed diffuse anaplasia, 2 cases of pleomorphic RMS showed focal anaplasia. Out of 3 cases of RMS- NOS, 2 exhibited focal anaplaisa and one displayed Diffuse anaplasia. Both Alveolar RMS had no features of anaplasia. Conclusion: Presence of Anaplasia is a frequent observation in pediatric RMS. Anaplasia is often under reported in pediatric RMS. Pathologist should be more aware of this rare phenomenon.

Presentación atípica de cáncer de tiroides en Hospital Escuela Universitario, Honduras / Atypical Thyroide Cancer, University School Hospital, Honduras

A las masas o protuberancias en la glándula tiroides se les llama nódulos tiroideos, la mayoría son benignos, pero alrededor de 2-3 de 20 son cancerosos. El carcinoma anaplásico, representa alrededor del 2%, es más frecuente en el sexo femenino (proporción 3:1). Objetivo: presentar un caso atípico de tumor canceroso en tiroides, técnicas diagnósticas y tratamiento aplicado. Presentación decaso clínico: paciente femenina de 51 años, procedente de Tegucigalpa, evangélica, ama de casa, con historia de masa en región anterior del cuello de un año de evolución, crecimiento progresivo, dolorosa a la palpación, que presentaba úlcera sangrante de un mes de evolución y 8 cm de diámetro. Examen físico: masa de 15 x 15 cm, móvil, asimétrica, bordes irregulares, consistencia dura, adherida a planos profundos. Exámenes complementarios: T4 libre 1.14 ng/dL, hormona estimulante tiroidea 2.10 mU/mL. Ultrasonido de cuello + Doppler:masa sólida, lóbulos aumentados de tamaño y con microvascularidad. Tomografía contrastada: masa en cartílago tiroides de 7.6 x 6.9 cm, bordes definidos, múltiples calcificaciones, sin áreas de necrosis, sin compresión de estructuras adyacentes; ganglios bilaterales aumentados de tamaño. Biopsia por Aspiración con Aguja Fina:carcinoma anaplásico de tiroides con focos de neoplasia papilar y variante de células altas. Tratamiento: tiroidectomía total, mostrando glándula tiroides aumentada de tamaño, peso aproximado de 200 g; levotiroxina 100 µg vía oral c/día de por vida, calcio 2 tabletas vía oral c/día por un mes. Conclusión: El estudio confirmatorio de cáncer anaplásico de tiroides es la biopsia por aspirado con aguja fina; sin embargo, la biopsia por disección, confiere un diagnóstico definitivo cuando existen dudas sobre la toma de muestra o cuando la clínica del paciente no corresponda con el diagnóstico de la biopsia por aspiración con aguja fina...(AU)

Progress in treatment of bilateral Wilms'tumor / 中国肿瘤临床

Bilateral Wilms'tumor (BWT) affects approximately 5% of children with WT. The goal of treatment in BWT is to preserve normal renal function as much as possible, in order to postpone and reduce the risk of end-stage renal disease and other long-term complications. For neoadjuvant chemotherapy, vincristine/dactinomycin/doxorubicin is used in cases with favorable histology. Cases with diffuse anaplasia or other unfavorable histology receive an intensified regimen, including additional agents such as ifosfamide, cy-clophosphamide, carboplatin, and others. Nephron-sparing surgery is performed to preserve long-term renal function. Radiotherapy is given according to local staging, when margins or lymph nodes are positive, or tumor spillage has occurred, or pathological type is ana-plasia. The survival rate of relapsed BWT is low. Some experts suggest that patients with recurrent BWT are most likely to benefit from high-dose therapy including stem-cell transplantation, but this remains controversial.

Supratentorial Pilocytic Astrocytoma Mimicking Convexity Meningioma with Early Anaplastic Transformation: A Case Report

Meningiomas and pilocytic astrocytomas are benign intracranial tumors. Pilocytic astrocytomas arises frequently at the posterior fossa in childhood. Meningiomas have several image findings, such as a dural tail sign, bony erosion, and sunburst appearance on angiography. However, pilocytic astrocytomas with these findings have been rarely reported. In this report, we describe a mass with typical image findings of a meningioma, but diagnosed as a supratentorial pilocytic astrocytoma with early anaplastic transformation.

Aspiration Cytology of the Osteoclastic Variant of Anaplastic Thyroid Carcinoma: with Special Emphasis on the Undifferentiated Mononuclear Cells

Anaplastic thyroid carcinoma (ATC) is an uncommon aggressive malignant tumor, and the osteoclastic variant of ATC is extremely rare. We report here on the fine needle aspiration cytology of the osteoclastic variant of ATC in an 83-year-old woman. The smear was composed of many oval to slightly elongated undifferentiated mononuclear cells admixed with multinucleated osteoclast-like giant cells. The mononuclear tumor cells revealed inconspicuous nuclear pleomorphism and the nuclei were characterized by vesicular chromatin and an indented or lobulated nuclear membrane with conspicuous nuclear grooves. A few epithelial clusters suggestive of a papillary carcinoma component were also observed. Making the proper cytological diagnosis of the osteoclastic variant of ATC is helpful to determine the proper treatment modality for these patients.

Anaplastic Ganglioglioma in a Middle-aged Woman: A Case Report with a Review of the Literature

We report a case of anaplastic ganglioglioma. A 45-yr-old woman was admitted with a 5-month history of headache and dizziness, both of which progressed slowly. Preoperative magnetic resonance imaging revealed a strong enhancing mass in the left frontal lobe extending to the cingulate gyrus. Adjuvant radiation therapy and chemotherapy were given after gross total resection of the tumor. Histological and immunohistochemical studies showed an anaplastic ganglioglioma. Gangliogliomas of the central nervous system are rather uncommon tumors, and anaplastic ones are extremely rare. The pertinent literature regarding gangliogliomas is reviewed.

Clinical and Pathophysiological Characteristics of Cerebral Gliomas without Enhancement on Magnetic Resonance Images

It is often assumed that a parenchymal brain lesion which shows no contrast enhancement on magnetic resonance imaging(MRI) is a"low-grade tumor". We investigated a series of 20 patients with nonenhancing cerebral gliomas ; all underwent stereotactic biopsy or open resection, and histological diagnoses were astrocytoma(n=7), anaplastic astrocytoma(n=7), oligodendroglioma(n=5), and ganglioglioma(n=1). Before surgery, 11 patients underwent [18F] fluorodeoxyglucose positron emission tomography(FDG-PET), and this showed hypermetabolic features in two of four patients with anaplastic astrocytomas and in one of seven with astrocytomas or oligodendrogliomas. Among 17 patients, the mean Ki-67 labeling index was 4.4+/-4.3(range : 0.1-16.7), and the standard error was 1.04. These results indicate that in cerebral gliomas, the enhancing pattern seen on MRI cannot predict the degree of malignancy, and we suggest that even if imaging is consistent with "low-grade glioma", histological verification and close follow-up of clinical course are both important.

Histopathological and Immunohistochemical Features of Wilms' Tumor

Wilms' tumor is one of the most common primary malignant tumors of the kidney during infancy and childhood and is known to be originated from the primitive cells of metanephric blastema. It presents difficulties when encountered in deciding the presence of anaplasia or in differentiating it from other renal tumors of childhood with different biologic behavior because of its diverse histologic patterns and varying degrees of differentiation. Evaluation of clinical and histopathologic features in terms of prognostication was done of 32 cases of Wilms' tumor which were surgically resected and diagnosed in the period from January 1979 through June 1992. Immunohistochemical reaction for cytokeratin, vimentin, actin and desmin was also analysed on all cases of Wilms' tumor in conjunction with clear cell sarcoma of the kidney(CCSK), malignant rhabdoid tumor of the kidney(MRTK) and congenital mesoblastic nephroma(CMN) to assess the validity of immunohistochemistry in differentiating Wilms' tumor from these renal tumors. Twenty four(75%) cases were diagnosed before the age of 5 and 40.7% were under 2 years old. Mixed type was most common(62.5%), followed by epithelial, blastemal and stromal predominant type in descending order of frequency. Anaplasia was observed in 3 cases(9.4%), two of which were epithelial predominant type and one blastemal predominant type. Treatment modality and presence of anaplasia were significantly correlated with 5 year survival rate of patients. Immunohistochemical stain revealed that all epithelial component of Wilms' tumor were positive for cytokeratin and 56.3% of Wilms' tumor had blastemal component which were positive for both cytokeratin and vimentin. Twenty cases(62.5%) of Wilms' tumor had blastemal component which were positive for cytokeratin with a proportion of more than 5% of reactive cells. Stromal component of Wilms' tumor generally did not show differentiation into the specialized type of tissue and all revealed positive reactions for vimentin among which some revealed positive reactions for actin. Only 3 out of 6 cases with rhabdomyoblastic differentiation were positive for desmin. CCSK, MRTK and CMN which have different biologic behavior and treatment modality compared to Wilm's tumor showed positivity only for vimentin and/or actin. In summary, treatment modality and presence of anaplasia are significantly correlated with patients' survival and the immunohistochemical stain for cytokeratin is very helpful in confirming the presence of blastemal component and useful in the differential diagnosis of Wilms' tumor from other kinds of pediatric renal tumors.

Oligodendroglioma in childhood

Fifteen pediatric (age under 16) cases of oligodendroglioma (ODG) were surgically proven from January 1985 to April 1992 at the Division of Pediatric Neurosurgery, Seoul National University Children's Hospital. To observe the proportion of ODG's in primary intracranial tumors, the location of ODG's and the prognostic significance of the histological grading of ODG's in childhood, the 15 cases of pediatric ODG's were retrospectively analyzed. ODG's accounted for 5.6% of pediatric primary intracranial tumors operated on during the same period. Nine tumors were located in the cerebral hemisphere (3 cases each in the frontal, temporal and parietooccipital lobes), 1 in the thalamus, 2 in the pons-medulla, 2 in the cerebellum and 1 in the thoracolumbar spinal cord. Four tumors were anaplastic and an additional case showed positive cerebrospinal fluid (CSF) cytology for tumor cells. All the cases of anaplasia or positive CSF cytology had a poor outcome. All the seven cases of benign ODG's in cerebral hemispheres presented with seizures which were controlled with or without medication after tumor removal.