Geethashree Mukherjee

Synovial sarcoma of the neck

Primary synovial sarcoma of the head and neck region is a rare tumor. This report describes seven cases of primary synovial sarcomas, of which two were in the parapharyngeal region, two in the supraclavicular region, and one each in the hypopharynx, sternocleidomastoid and submandibular regions. Clinical presentations, radiological findings, histopathology and management are reviewed. All patients received multimodal therapy using aggressive surgery, radiotherapy and chemotherapy. Five of the patients are alive and disease free after 24–108 months of follow-up. Achievement of locoregional control appears to be the hallmark of successful therapy.

Rhabdomyosarcoma of the tongue

Although rhabdomyosarcoma (RMS) has a predilection for the head and neck region its occurrence in the tongue is uncommon. We report 2 cases of RMS of the tongue, 1 paediatric and 1 adult patient. The child who had RMS of the alveolar type involving anterior two-thirds of the tongue was treated with surgery and chemotherapy and is disease-free at 84 months of follow-up. The adult patient had locally extensive embryonal RMS of posterior third of the tongue, received chemotherapy and radiotherapy but died with progressive disease at 24 months of follow-up.

Primitive neuroectodermal tumor of the kidney. A report of two cases diagnosed by fine needle aspiration cytology.

BACKGROUND Primitive neurocetodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectrodermal origin most often presenting as bone or soft tissue masses. There are very few reported cases of PNET of the kidney and none diagnosed by fine needle aspiration cytology (FNAC), to the best of our knowledge, in the world literature. We present two cases of renal PNET diagnosed on cytology. CASES Two patients with renal masses were diagnosed as having PNET on FNAC. Cytologically the tumors showed a dispersed population of malignant small round cells with focal rosette formation and perivascular arrangement of tumor cells. Immunohistochemistry on the cell blocks in both cases showed strong membrane positivity for CD99 (MIC2). Cytogenetic studies in both cases showed the characteristic t(11;22)(q24;q12) translocation, with additional chromosomal abnormalities in case 2. CONCLUSION PNET of the kidney is a distinct entity and can be diagnosed on fine needle aspiration smears and confirmed with immunohistochemistry and cytogenetic studies. A diagnosis of PNET must be included in the differential diagnosis of renal masses in adolescents and young adults.

Rhabdomyosarcoma: cytogenetics of five cases using fine-needle aspiration samples and review of the literature

Cytogenetic analyses of fine-needle aspiration samples were performed on five cases of which three were alveolar rhabdomyosarcomas (RMS), one was embryonal RMS and one was RMS of mixed alveolar and embryonal histology. Three cases of alveolar RMS and one case of embryonal RMS showed t(2;13). A del(1)(p11) in a mixed alveolar and embryonal RMS was observed without the presence of t(2;13). add(17)(q25) was present in one of the alveolar RMS along with a t(2;13). Modal number of chromosome in the five cases ranged from hyperdiploid to hypertetraploid. Clinical, cytological, histopathological and cytogenetic findings are correlated. The role of additional abnormalities is discussed with a review of appropriate literature.

Submandibular synovial sarcoma with t(X;18) and synovial sarcoma of the toe with additional cytogenetic abnormalities: presentation of two cases and review of the literature

We report cytogenetic findings from fine-needle aspiration samples of two synovial sarcoma patients. The cases are of interest because (1) one case is of a rare site (submandibular region) of the head and neck, and (2) the other is a patient with synovial sarcoma of the toe showing additional cytogenetic abnormalities along with t(X;18). The literature of this tumor is reviewed.

Hepatoblastoma: Experience from a single center

BACKGROUND The cornerstones of successful treatment of hepatoblastoma (HB) include preoperative chemotherapy followed by complete anatomical resection of tumor, followed by chemotherapy. Advances in chemotherapy in the last 2 decades have been associated with a higher rate of tumor response and possibly a greater potential for resectability. AIMS We analyzed our single center experience with neoadjuvant chemotherapy (NACT) and surgery in HBs. SETTINGS AND DESIGN Our study included all children with HBs who received NACT and underwent surgical excision from January 1997 to July 2004. MATERIALS AND METHODS Patient characteristics, clinical features, clinical course, treatment modalities, and long-term outcome were analyzed. RESULTS There were 9 boys and 3 girls, aged 5-60 months (median age at tumor diagnosis was 24 months). All received NACT containing cisplatin and doxorubicin. Of the 12 children, 9 underwent hepatectomy and among them, 4 patients each had right and left hepatectomy and 1 patient underwent right extended hepatectomy. After surgery, all patients completed rest of the chemotherapy course (total 6 cycles). R0 resection was carried out in all the 9 cases with no life-threatening complications. CONCLUSIONS Our experience of the 9 cases, although less in number, reaffirms the advantages of NACT followed by surgery. The prognosis for patients with resectable tumors is fairly good in combination with chemotherapy.

Renal angiomyolipoma - sarcomatous transformation and cavoatrial extension.

Renal Angiomyolipoma occurs both sporadically and in association with tuberous sclerosis [1]. Earlier regarded as a hamartoma, angiomyolipoma is now believed to be neoplastic, showing clonal non random inactivation of the X chromosome [2]. Though generally regarded as benign, angiomyolipomas rarely produce complicated clinical courses that can be life threatening. It is a common cause of severe perirenal and retroperitoneal haemorrhage [3].More rarely they display malignant potential with rapid growth and metastasis. These malignancies associated with angiomyolipoma include malignant epithelioid angiomyolipomas and sarcomatous transformation of one of the components of the tumor, most commonly leiomyosarcoma [3–6]. Extrarenal growth and vascular invasion are rare [7].