Adrenal Leiomyosarcoma Presenting with Chest Wall Nodule: a Histological Insight in an Intriguing Case
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CASE REPORT
Adrenal Leiomyosarcoma Presenting with Chest Wall Nodule: a Histological Insight in an Intriguing Case Purnima Bharti 1 & Ravi H. Phulware 1 & Arvind Ahuja 1
&
Aneesha Mohanpuria 1 & Hemant Goel 2 & Minakshi Bhardwaj 1
Received: 27 March 2020 / Accepted: 7 May 2020 # Indian Association of Surgical Oncology 2020
Introduction Mesenchymal tumours of adrenal gland are rare. Malignant tumours are even rarer and include angiosarcoma and leiomyosarcoma. Primary adrenal leiomyosarcoma (PAL) is an extremely rare mesenchymal tumour and originates from the smooth muscle wall of the central adrenal vein and its branches [1, 2]. Leiomyosarcoma of the adrenal gland is most often found in elderly individuals. To the best of our knowledge, it has been reported only in 35 patients in the English literature [2]. In the present case, we report another case of primary adrenal leiomyosarcoma and review the clinical and pathological characteristics, along with its rarity of chest wall metastasis. Because this entity is rarely encountered, there is the possibility that diagnosis of the disease is delayed because most clinicians would not be familiar with its presentation, diagnosis and management. Its diagnosis is based entirely on the findings of both histological and immunohistochemical * Arvind Ahuja [email protected] Purnima Bharti [email protected] Ravi H. Phulware [email protected] Aneesha Mohanpuria [email protected] Hemant Goel [email protected] Minakshi Bhardwaj [email protected] 1
Department of Pathology, PGIMER, ABVIMS, RML Hospital, New Delhi, India
2
Department of Urology, PGIMER, ABVIMS, RML Hospital, New Delhi, India
(IHC) evaluations, which are indispensable not only for determining the tumour type but also for predicting the biological behaviour [1–3].
Case History A 60-year-old female came to hospital with complains of dull flank pain and abdominal fullness for 3 months. On examination, an abdominal mass was palpable in the left lumbar region. Ultrasound and magnetic resonance imaging (MRI) of abdomen showed a retroperitoneal mass roughly measuring 5 × 4 × 2 cm originating from adrenal gland without any extension (Fig. 1a). Uterus and bilateral ovaries were normal. Twentyfour-hour urine for vanillylmandelic acid, metanephrine, and nor-metanephrine as well as urinary free cortisol was normal. On laparoscopy, there was no gross sign of infiltration, and the tumour was completely removed in compliance with the principles of radical surgery. Her postoperative recovery was unremarkable. Gross examination of the specimen showed 5 × 3 × 2 cm mass weighing 132 g with cut surface revealing a wellcircumscribed grey white tumour with solid and firm areas and typical whorling pattern (Fig. 1b). Microscopic examination of the mass showed tumour comprising of spindle cells arranged in intersecting fascicles, mild to moderately pleomorphic blunt ended nuclei, moderate amount of eosinophilic cytoplasm and mitosis up to 5-6 per 10 high power field (HPF) (Fig. 1c and d) and evidence of tum
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