Seethalakshmi Viswanathan

The cytological diagnosis of paediatric renal tumours

Fine needle aspiration cytology (FNAC) is used for preoperative diagnosis of paediatric renal tumours, especially in centres where preoperative chemotherapy is advocated in Wilms’ tumour. This review focuses on salient cytological features in specific paediatric renal tumours, the approach to resolving a differential diagnosis and the role of ancillary methods in diagnosis of paediatric renal tumours. Crucial differential diagnoses include distinguishing: Wilms’ tumour from benign tumours in the kidney like multicystic nephroma or congenital mesoblastic nephroma; aggressive non-Wilms’ tumours of kidney like rhabdoid tumour of kidney; and Wilms’ tumour from other paediatric round cell sarcomas like neuroblastoma, non-Hodgkin lymphoma etc. An approach based on classifying smears according to their cellular patterns as triphasic, round cell, spindle cell or epithelioid cell type assists in classifying paediatric renal tumours on cytology. Immunocytochemistry for WT1, cytokeratin, synaptophysin, leucocyte common antigen and MIC2 will aid in evaluating round cell tumours in the renal region, while WT1, bcl2, vimentin and desmin will be useful for spindle cell tumours in that region. Extra material can also be evaluated for demonstration of specific cytogenetic abnormalities in these tumours. A checklist of common tumours in a particular age group, relevant clinical information, awareness of distinctive and overlapping cytological features, and appropriate use of immunocytochemistry with cytogenetics go a long way in ensuring an accurate cytological diagnosis. Used judiciously, FNAC is as effective a tool as a core biopsy for preoperative diagnosis of paediatric renal tumours, and with experience a 92% accuracy rate can be achieved.

Orbital medulloepitheliomas—With extensive local invasion and metastasis: A series of three cases with review of literature

Intraocular medulloepithelioma is a rare embryonal neoplasm which usually presents in childhood as a ciliary body mass. This tumor is usually confined to the orbit and rarely tends to be locally aggressive and metastatic. We describe three rare cases of metastatic malignant teratoid medulloepithelioma. The patients were males aged 7, 16 and 25 years with right eye involvement in all the cases. All patients had local recurrence (multiple in two) and metastasis to the intraparotid and cervical lymph nodes. Distinction from round cell tumors and primary salivary gland neoplasms occurring in the parotid gland is strongly warranted, especially in biopsy samples, because the biological behaviour and treatment of medulloepithelioma differs greatly from the above lesions.

Metastatic papillary carcinoma of thyroid masquerading as a renal tumour.

Differentiated papillary carcinomas constitute 80–90% of thyroid malignancies and are known for regional nodal metastasis.1 Only 2% of patients present with haematogenous metastases involving lungs, brain and bone.2 A man in his 50s presented elsewhere with flank pain and haematuria. Computed tomography scan revealed a 5 cm cystic, necrotic mass in the upper pole of the right kidney. Following a right radical nephrectomy for a primary renal malignancy the patient was referred to our institute. Gross examination of a kidney measuring 8.5×5×5 cm revealed a 5×5×4 cm mass, with gelatinous brown areas, adherent to the renal capsule in the …

Primary PNET of maxilla: an unusual presentation.

Primitive neuroectodermal tumors (PNETs) are a type of small round cell tumors developing from migrating embryonal cells of the neural crest. Peripheral primitive neuroectodermal tumors (pPNETs) are less common with varying incidence of occurrence in head and neck region. Only 8 reported cases of primary PNET of maxilla are available in English literature. We report a case of 8-year-old boy diagnosed as pPNET of maxilla after detailed radiologic, histopathologic, including immuno-histochemical examination and molecular diagnosis using reverse transcription-polymerase chain reaction showing EWS-FLI1 translocation. The boy was treated with multiagent combination chemotherapy to be followed by definitive radiation therapy. A brief literature review of diagnosis and management of the previous 8 reported cases is done. In view of no definitive guideline for management of such cases, treatment on the lines of other pPNET is suggested.

A study of pathologic risk factors in postchemoreduced, enucleated specimens of advanced retinoblastomas in a developing country.

CONTEXT Advanced cases of retinoblastoma are treated with chemoreduction followed by enucleation. Further adjuvant therapy is recommended in patients with known pathologic risk factors (PRFs). OBJECTIVES To determine the PRFs in enucleated specimens after chemoreduction and their association for adverse events of recurrence, metastasis, or death. DESIGN This was a retrospective study of 77 enucleation specimens from patients treated between January 2000 and September 2008 with prior chemoreduction that were accessioned in the pathology department of a tertiary referral cancer center with an average follow-up of 24 months. Various PRFs were noted and their association with the development of an adverse event was recorded. RESULTS Of 77 patients, (male to female ratio, 51∶26), the incidence of overall PRF was 51.9%, and retrolaminar optic nerve invasion (32.5%), optic nerve cut margin (12.9%), massive choroidal invasion (26%), scleral invasion (23.4%), vitreous seedings (44.2%), and anterior segment invasion (20.8%). Undifferentiated tumor (>60%) was seen in 60.3% of cases (41 of 68 patients with differentiation available). Adverse event occurred in 18 of 72 patients with available follow-up (25%). Retrolaminar optic nerve invasion, optic nerve cut margin involvement, and scleral invasion were independent prognostic factors predicting the occurrence of an adverse event. Undifferentiated tumor (>60%) was a significant risk factor in univariate analysis, which is the unique feature in this study. CONCLUSIONS Classic PRF with the addition of a predominant presence from the undifferentiated component were associated with adverse outcomes in retinoblastoma treated with anterior chemotherapy. The latter may represent chemoresistant clones and more intensive adjuvant chemotherapy may be warranted in these patients.

Squamous Differentiation in a Sarcomatoid Chromophobe Renal Cell Carcinoma An Unusual Case Report With Review of the Literature

We describe an extremely rare occurrence of a squamous differentiation in a sarcomatoid chromophobe renal cell carcinoma in a 45-year-old woman with nodal and lung metastasis at presentation. The tumor on histology showed all 3 components intimately admixed with each other, which to the best of our knowledge is the first such case to be reported in the literature. The renal pelvis was smooth walled and uninvolved. Kidney-specific cadherin was positive in the chromophobe renal cell carcinoma areas and negative in the sarcomatoid and squamous areas.

Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature

Cutaneous angiosarcoma or lymphangiosarcoma represents an uncommon aggressive tumor known to arise on a background of chronic lymphedema secondary to various etiologies, principally following surgery or irradiation. There have been rarely reported cases of angiosarcoma following infective conditions that eventuate with lymphatic stasis. We report a case of angiosarcoma arising after 33 years within a background of filariasis. Awareness of this association can lead to early diagnosis and appropriate treatment of this potentially fatal malignant tumor.

Parvovirus B19 presenting with persistent pancytopenia in a patient of T-ALL post induction chemotherapy diagnosed on bone marrow examination

Manifestations of parvovirus B19 vary even in the normal host from asymptomatic or subclinical infection to a spectrum of illness with symptoms during viremic and immune complex mediated stage of disease. We report the morphological findings of parvovirus B19 infection (confirmed on serology) in a patient of T-acute lymphoblastic lymphoma (T-ALL) who underwent induction phase of chemotherapy (MCP 842 protocol). Persistent pancytopenia in the bone marrow aspirate with mild increase in blasts was thought to be due to failure to achieve marrow remission. However, giant pronormoblasts with prominent intranuclear inclusions confirmed on trephine biopsy led to the suspicion of parvovirus B19 infection which was later confirmed on serology. This case is presented to report the rarely seen classical morphological feature of parvovirus infection on bone marrow examination which was incidentally the first investigation to diagnose the viremic phase of the infection, indicating that a high index of suspicion needs to be kept in mind while examining bone marrows of susceptible patients.

Diagnostic dilemmas in fine needle aspiration cytology of an ectopic kidney: a case report.

BACKGROUND Congenital anomalies are easily diagnosed on radiology and rarely have an unusual presentation requiring an invasive diagnostic procedure. Fine needle aspiration cytology (FNAC), though a well-established diagnostic technique for abdominal and retroperitoneal lesions, is fraught with several challenges and pitfalls. CASE We report a case of a 39-year-old woman with an ectopic kidney presenting as an abdominal mass. On FNAC the cellular aspirate was misinterpreted as a paraganglioma or metastatic carcinoma. CONCLUSION This case highlights the fact that on FNAC, normal cellular components, especially from the kidney, provide a significant pitfall for overdiagnosis, potentially resulting in unnecessary surgical explorations.