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Journal Thoracic Oncology

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Abstracts <strong>Journal</strong> of <strong>Thoracic</strong> <strong>Oncology</strong> • Volume 12 Issue S1 January 2017<br />

POSTER SESSION 2 – P2.04: MESOTHELIOMA/THYMIC MALIGNANCIES/ESOPHAGEAL<br />

CANCER/OTHER THORACIC MALIGNANCIES<br />

ESOPHAGEAL CANCER AND OTHER MALIGNANCIES –<br />

TUESDAY, DECEMBER 6, 2016<br />

P2.04-035 SURGICAL PERPLEXITIES IN A RARE CASE OF<br />

SYMPTOMATIC MEDIASTINAL LYMPHANGIOMA<br />

Kingsfield Ong, Keerthi Rajapaksha, Aneez Ahmed<br />

Tan Tock Seng Hospital, Singapore/Singapore<br />

Background: Mediastinal lymphangiomas are very rare and are often<br />

found in close proximity to vital structures of the head and neck. Thus,<br />

the surgical approach can be challenging. It is debatable to employ open<br />

surgery techniques via a trap-door incision or median-sternotomy in attempt<br />

to achieve complete surgical resection for a benign lesion. We report a<br />

case of symptomatic mediastinal lymphangioma that was successfully<br />

managed with a Video Assisted Thoracoscopic Surgery (VATS) approach.<br />

Methods: A 28-year-old female with no significant past medical history was<br />

found to have a superior mediastinal mass on a chest radiograph whilst<br />

undergoing investigations for acute left-sided upper back pain. Computed<br />

tomography (CT) study of the thorax revealed a large lobulated soft tissue<br />

mass in the left superior mediastinum, extending to the supraclavicular<br />

region, measuring approximately 4.4 x 4.9 x 7.4 cm, encasing the left<br />

carotid, subclavian and vertebral arteries, and the left internal jugular and<br />

brachiocephalic veins. Ultrasound guided core biopsy was inconclusive.<br />

Results: The patient underwent surgery via left VATS approach. Frozen<br />

section of the lesion revealed partial lymphoid infiltrates and scattered<br />

cystic-like spaces. No atypical cells or malignancy was seen. Maximal<br />

debulking of the lesion was performed with the aid of bipolar diathermy and<br />

harmonic ultrasonic energy devices instead of a complete resection. The<br />

patient made an uneventful recovery and was discharged home on the first<br />

post-operative day. Histological examination showed lymphoid aggregrates<br />

and dilated lymphatic spaces with a rim of smooth muscle that are typical of<br />

lymphangiomas. She remained well and pain free at her 1yr follow-up clinic.<br />

Conclusion: In conclusion, due to the unique nature and characteristics of<br />

mediastinal lymphangiomas, pre-operative diagnosis and treatment are<br />

challenging. Minimally invasive techniques for maximum debulking are useful<br />

for symptomatic treatment as complete resection may not always be possible.<br />

Keywords: video assisted thoracoscopic surgery, lymphangioma<br />

Conclusion: Primary dedifferentiated liposarcoma of the mediastinum is<br />

extremely rare. Chemotherapy and radiotherapy seems to be ineffective;<br />

radical surgical resection represents the best therapeutic modality. A closed<br />

and long-term follow-up is required because of the high risk of recurrence.<br />

Keywords: mediastinum, liposarcoma<br />

POSTER SESSION 2 – P2.04: MESOTHELIOMA/THYMIC MALIGNANCIES/ESOPHAGEAL<br />

CANCER/OTHER THORACIC MALIGNANCIES<br />

ESOPHAGEAL CANCER AND OTHER MALIGNANCIES –<br />

TUESDAY, DECEMBER 6, 2016<br />

POSTER SESSION 2 – P2.04: MESOTHELIOMA/THYMIC MALIGNANCIES/ESOPHAGEAL<br />

CANCER/OTHER THORACIC MALIGNANCIES<br />

ESOPHAGEAL CANCER AND OTHER MALIGNANCIES –<br />

TUESDAY, DECEMBER 6, 2016<br />

P2.04-036 GIANT PRIMARY DEDIFFERENTIATED LIPOSARCOMA<br />

OF THE ANTERIOR MEDIASTINUM: AN EXTREMELY RARE<br />

OCCURRENCE<br />

Luigi Ventura 1 , Letizia Gnetti 2 , Cesare Braggio 1 , Valeria Balestra 1 , Paolo<br />

Carbognani 1 , Enrico Maria Silini 2 , Michele Rusca 1 , Luca Ampollini 1<br />

1 Department of Surgical Sciences, <strong>Thoracic</strong> Surgery, University Hospital of Parma,<br />

Parma/Italy, 2 Pathological Anatomy and Histology, University Hospital of Parma,<br />

Parma/Italy<br />

Background: To present an extremely rare case of giant primary<br />

dedifferentiated liposarcoma of the anterior mediastinum. Methods: A<br />

70-year-old male presented for persistent dyspnoea. Chest CT-scan showed a<br />

huge well-defined solid mass with a fatty tissue component in the<br />

anterosuperior mediastinum (Fig.1A). The tumour caused a wide displacement<br />

of the right lung and mediastinal organs with no signs of invasion. PET-CT scan<br />

showed a unique uptake of the mediastinal mass (Suv max=4.6). A surgical<br />

resection was proposed. A right emiclamshell incision was performed. On<br />

exploration, a huge mass of the anterior mediastinum causing the compression<br />

of the right lung, pericardium and great vessels was observed; no sign of<br />

adjacent organs invasion was found. The tumour was radically resected and the<br />

patient was discharged uneventfully after 6 days. The patient denied any<br />

adjuvant therapy. No evidence of recurrence has been identified as of 16 months<br />

postoperatively. Results: Macroscopically, the smooth, lobulated, well<br />

demarcated, yellowish-white mass weighed 2102g and measured 27x23x13cm<br />

(Fig.1B). Microscopically, the tumour presented a dedifferentiated component<br />

characterized by spindle cells organized in a fascicular pattern with abundant<br />

stroma and necrosis (Fig.1C) and a second component of lipoma-like welldifferentiated<br />

liposarcoma (Fig.1D). Figure 1E showed both components. The<br />

spindle cells displayed a moderate degree of nuclear atypia, with moderate to<br />

marked levels of pleomorphism and cellularity. The spindle cells resulted<br />

positive for desmin and SMA and had an elevated proliferative index<br />

(Ki67=50%) (Fig.1F). Immunohistochemically, tumour cells of both components<br />

were positive for CDK4 (Fig.1G) MDM2 (Fig.1H) and negative for myoglobin,<br />

EMA, S-100. Given that, a final pathological diagnosis of primary<br />

dedifferentiated liposarcoma of the mediastinum was disclosed.<br />

P2.04-037 SOLITARY FIBROUS TUMOR OF THE PLEURA<br />

ASSOCIATED WITH SEVERE HYPOGLICEMIA: THE DOEGE-POTTER<br />

SYNDROME<br />

Luigi Ventura 1 , Letizia Gnetti 2 , Cesare Braggio 1 , Paolo Carbognani 1 , Michele<br />

Rusca 1 , Enrico Maria Silini 2 , Luca Ampollini 1<br />

1 Department of Surgical Sciences, <strong>Thoracic</strong> Surgery, University Hospital of Parma,<br />

Parma/Italy, 2 Pathological Anatomy and Histology, University Hospital of Parma,<br />

Parma/Italy<br />

Background: to present an extremely rare case of Doege-Potter Syndrome<br />

(DPS). Methods: a 66 years-old man, non-smoker, was admitted for<br />

disorientation and confusional state. Blood tests showed a severe<br />

hypoglycemia (30 mg/dl); the patient was not diabetic and did not take<br />

antihyperglycemic agents. An extremely low values of insulin and C-peptide ​<br />

was also found. A chest CT-scan showed a 20x16cm lobulated mass occupying<br />

the right hemithorax (Figure 1A). FDG-PET showed a unique uptake of the<br />

lesion (SUVmax 2.5). After a multidisciplinary discussion, a surgical resection<br />

was proposed. The lesion of pleural origin was radically resected through a<br />

right posterolateral thoracotomy. From the 1st postoperative day, the blood<br />

glucose levels were normal; the patient was discharged on the 5th<br />

postoperative day uneventfully. Results: the mass measured 26x18x12cm,<br />

presented polylobate margins, elastic consistency and grayish-white color<br />

(Figure 1B). Microscopically, a mesenchymal neoplasm characterized by<br />

spindle cells with moderate atypia, hyperchromasia and nuclear<br />

pleomorphism, organized in a fascicular pattern was found (Figure 1C). A<br />

moderate deposition of collagen extracellular matrix, with areas of hyaline<br />

involution and foci of necrosis was evident (Figure 1D). The mitotic index was<br />

9x10 HPF. Immunohistochemically, tumor cells were positive for CD34, CD99,<br />

BCL-2, vimentin, desmin, IGF1-R, IGF-2, Ki67 (15%) and negative for EMA,<br />

smooth muscle actin, cytokeratin pool, c-kit, DOG-1, myogenin, calretinin,<br />

S100. Considering these histopathologic features, a diagnosis of malignant<br />

solitary fibrous tumor of the pleura was made. After 18 months, the patient is<br />

in good condition and free of disease.<br />

S534 <strong>Journal</strong> of <strong>Thoracic</strong> <strong>Oncology</strong> • Volume 12 Issue S1 January 2017

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