ABSTRACT
Background: Extracranial head and neck schwannomas are rare tumors which are often clinically misdiagnosed. Preoperative diagnosis of these tumors is challenging but imperative for surgeons so as to avoid nerve damage during excision. Materials and Methods: Sixteen patients diagnosed with extracranial head and neck schwannomas over a period of 2 years were retrospectively analyzed. Clinical details including preoperative fine-needle aspiration cytology (FNAC) and/or computed tomography (CT)/magnetic resonance imaging (MRI) findings were retrieved. FNAC smears and formalin-fixed paraffin-embedded sections were evaluated. Results: Among these 16 tumors, 6 (37.5%) were located in the lateral aspect of neck, 4 (25%) in scalp, 2 (12.5%) in orbit, and one each (6.25%) in palate, tongue, submandibular gland and parotid gland. The mean patient age was 31.3 years. FNAC was performed in 14 cases, of which 8 cases (58.3%) showed features of benign nerve sheath tumor (BNST), two cases (14.2%) were inconclusive with possibility of mesenchymal lesion, two cases (14.2%) were inadequate, one case (8.3%) showed features suggestive of schwannoma, and a diagnosis of nerve sheath tumor inconclusive for malignancy was rendered in a single case. The sensitivity of FNAC in diagnosis of BNST was 71.4%. CT or MRI was performed in five cases, of which an accurate diagnosis was rendered only in one case of orbital schwannoma. Conclusion: Imaging has a limited role in the preoperative diagnosis of head and neck schwannomas owing to nonspecific radiological features. Cellular aspirate smears are helpful in accurate diagnosis even at unusual locations
ABSTRACT
Mycosis fungoides is a cutaneous T-cell lymphoma with a high risk for developing secondary malignancies, especially B-cell lymphoproliferative disorders. About 40 cases of Hodgkin's lymphoma associated with mycosis fungoides have been reported in literature till date. We report a case of a 35-year-old gentleman who presented with intensely itchy reddish lesions all over the body. Multiple skin biopsies taken from the lesions on scalp and back confirmed the clinical diagnosis of mycosis fungoides. While on treatment, he presented with multiple bilateral cervical, axillary and inguinal lymphadenopathy 9 years after the primary diagnosis of mycosis fungoides. Excision biopsy of a cervical lymph node revealed partial effacement of architecture by a tumor comprising polymorphous background. Histopathology and immunohistochemistry revealed a diagnosis of Hodgkin's lymphoma - nodular sclerosis subtype. The patient was started on chemotherapy for stage IV Hodgkin's lymphoma. Our case emphasizes the importance of keeping secondary Hodgkin's lymphoma in mind while dealing with a patient of mycosis fungoides. Our case immunohistochemically supports the distinct etiopathogenesis of Epstein–Barr virus-negative Hodgkin's lymphoma vis-à-vis cutaneous mycosis fungoides.
ABSTRACT
Primary rhabdomyosarcoma (RMS) of the heart is a rare malignant tumor which has poor prognosis and survival despite surgery and adjuvant chemotherapy. The preoperative diagnosis is often difficult in view of nonspecific clinicoradiological findings. This report describes a case of a 60‑year‑old woman who was clinically diagnosed as left atrial myxoma. A diagnosis of pleomorphic RMS was made on histopathology after excision. Our case discusses the clinicopathological features and treatment options of cardiac RMS emphasizing the fact that histopathology and immunohistochemistry are essential to confirm the diagnosis of such an aggressive malignant tumor.
ABSTRACT
Background & objectives: Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms’ tumour (commonest tumour) from non-Wilms’ tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms’ tumour-1 protein (WT1) in paediatric renal tumours. Methods: A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study. WT1 immunostaining was done using mouse monoclonal WT1 antibody (clone: 6F-H2). Results: Of the 53 cases, 38 (72%) were of Wilms’ tumour. Non-Wilms’ group (15) included six cases of mesoblastic nephroma (MN), two each of clear cell sarcoma (CCSK), renal cell carcinoma (RCC) and peripheral neuroectodermal tumour (PNET) and one each of angiomyolipoma (AML), rhabdomyosarcoma (RMS) and malignant rhabdoid tumour (MRT). Proportion of WT1 positivity in Wilms’ tumour was 100 per cent in contrast to 26.7 per cent in non-Wilms’ tumours (P<0.001). Epithelial and blastemal components of Wilms’ tumour showed moderate (2+) nuclear and cytoplasmic staining in 80 (24/30) and 75 per cent (24/32) cases, respectively. MN, PNET, CCSK and AML were negative for WT1. RMS, RCC and MRT showed cytoplasmic staining, strongest in RMS. No significant association was seen between WT1 expression and NWTSG (National Wilms’ Tumor Study Group) stage. Interpretation & conclusions: WT1 helps to differentiate Wilms’ tumour from other paediatric renal tumours. It may help in differentiating the two subgroups of Wilms’ tumour which have distinct molecular pathogenesis and biological behaviour, however, further prospective studies are required for validation of this hypothesis.
ABSTRACT
Extraskeletal myxoid chondrosarcoma is a rare soft-tissue sarcoma, mostly occurring in the proximal extremities and limb girdle. Majority of the patients are in fifth and sixth decades of life with male preponderance. We report here a case of primary extraskeletal myxoid chondrosarcoma of the uterine adnexa involving the broad ligament and fallopian tube synchronously without any evidence of uterine/ovarian involvement in a young multiparous female of 27 years. After the histopathological diagnosis, re-excision of the tumor bed with wide local margins was recommended. Since the tumor has an aggressive course, with propensity for late recurrence and metastases to lungs, the patient must be considered for long-term follow-up.