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Low grade fibromyxoid sarcoma of the falciform ligament: a case report.

Harish K, Ashok AC, Alva NK - BMC Surg (2003)

Bottom Line: Very few cases of intra-abdominal location have been reported.We report a 37 year old man who presented with an abdominal mass and dragging pain.Pre-operative imaging suggested the possibility of a subcapsular hemangioma of liver.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgical Oncology, MS Ramaiah Medical College, Bangalore, India. drkhari@yahoo.com

ABSTRACT

Background: Low grade fibromyxoid sarcomas (LGFMS) are very rarely seen. They commonly arise from deep soft tissues of the lower extremities. Very few cases of intra-abdominal location have been reported.

Case presentation: We report a 37 year old man who presented with an abdominal mass and dragging pain. Pre-operative imaging suggested the possibility of a subcapsular hemangioma of liver.

Conclusions: Laparoscopy was useful to locate the tumor as arising from falciform ligament and made the subsequent surgery simpler. This is one of the large fibromyxoid sarcomas to be reported.

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Specimen showing tumor covered with falciform ligament and the tied ligamentum teres which is indicated by bold arrow (posterior view).
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Figure 4: Specimen showing tumor covered with falciform ligament and the tied ligamentum teres which is indicated by bold arrow (posterior view).

Mentions: A pre-operative fine needle aspiration was not done as there was a suspicion of hemangioma. A diagnostic laparoscopy was planned to visualize the location of the tumor and its relation to other organs. It was decided that the surgical plan would be based on the laparoscopic findings. If the lesion were found to arise from the liver or if the excision of the tumor necessitated removal of a segment of liver, it was decided to biopsy the lesion and plan a definitive surgery later. Laparoscopy revealed a large mass lesion situated between the leaves of the falciform ligament abutting against the anterior surface of the liver; but the liver per se was normal and free from the tumor. A number of enlarged tortuous blood vessels were seen running from the anterior abdominal wall to the mass. Anterior abdominal wall was incised and the tumor accessed through a supra-umbilical incision. The laparoscope also assisted in identifying the tortuous vessels limiting the intra-operative blood loss. Wide excision was completed. As it was desirable to perform an excision with intact capsule, a laparoscopic excision was not attempted. The globular bosselated well encapsulated mass [Fig 3, 4] measuring 21 × 16 × 10 cm was situated between the leaves of the falciform ligament with neither involvement of the abdominal wall nor the liver. A frozen section study was not considered as the surgery would not have been different if the report were to be either benign or malignant. Cut section showed a grayish white appearance with focal areas of hemorrhage [Fig 5]. Post operative recovery was uneventful. Histologically, on initial evaluation, the mass appeared to be composed of interlacing fascicles of spindle shaped fibroblasts with cartwheel arrangement [Fig 6, 7] suggesting a possibility of fibrous histiocytoma. However, further evaluation with multiple sections showed a tumor with moderate to low cellularity. The tumor was composed of bland spindle shaped cells with small hyperchromatic oval to tapering nuclei, containing finely clumped chromatin with palely eosinophilic cytoplasm. The cells showed mild nuclear pleomorphism with little mitotic activity. The cells were deposited in a variably fibrous and myxoid stroma having stellate cells [Fig 8]. The cells showed whorled arrangement in a random manner. Perivascular hypercellularity was noted in some areas [Fig 9]. Based on these features, the tumor was diagnosed as low grade fibromyxoid sarcoma. Immunohistochemistry was performed to confirm the diagnosis. The neoplasm stained strongly and diffusely for vimentin [Fig 10]. Staining for other markers including CD 34, CD 68, smooth muscle actin and S 100 were negative [Fig 11].


Low grade fibromyxoid sarcoma of the falciform ligament: a case report.

Harish K, Ashok AC, Alva NK - BMC Surg (2003)

Specimen showing tumor covered with falciform ligament and the tied ligamentum teres which is indicated by bold arrow (posterior view).
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC222986&req=5

Figure 4: Specimen showing tumor covered with falciform ligament and the tied ligamentum teres which is indicated by bold arrow (posterior view).
Mentions: A pre-operative fine needle aspiration was not done as there was a suspicion of hemangioma. A diagnostic laparoscopy was planned to visualize the location of the tumor and its relation to other organs. It was decided that the surgical plan would be based on the laparoscopic findings. If the lesion were found to arise from the liver or if the excision of the tumor necessitated removal of a segment of liver, it was decided to biopsy the lesion and plan a definitive surgery later. Laparoscopy revealed a large mass lesion situated between the leaves of the falciform ligament abutting against the anterior surface of the liver; but the liver per se was normal and free from the tumor. A number of enlarged tortuous blood vessels were seen running from the anterior abdominal wall to the mass. Anterior abdominal wall was incised and the tumor accessed through a supra-umbilical incision. The laparoscope also assisted in identifying the tortuous vessels limiting the intra-operative blood loss. Wide excision was completed. As it was desirable to perform an excision with intact capsule, a laparoscopic excision was not attempted. The globular bosselated well encapsulated mass [Fig 3, 4] measuring 21 × 16 × 10 cm was situated between the leaves of the falciform ligament with neither involvement of the abdominal wall nor the liver. A frozen section study was not considered as the surgery would not have been different if the report were to be either benign or malignant. Cut section showed a grayish white appearance with focal areas of hemorrhage [Fig 5]. Post operative recovery was uneventful. Histologically, on initial evaluation, the mass appeared to be composed of interlacing fascicles of spindle shaped fibroblasts with cartwheel arrangement [Fig 6, 7] suggesting a possibility of fibrous histiocytoma. However, further evaluation with multiple sections showed a tumor with moderate to low cellularity. The tumor was composed of bland spindle shaped cells with small hyperchromatic oval to tapering nuclei, containing finely clumped chromatin with palely eosinophilic cytoplasm. The cells showed mild nuclear pleomorphism with little mitotic activity. The cells were deposited in a variably fibrous and myxoid stroma having stellate cells [Fig 8]. The cells showed whorled arrangement in a random manner. Perivascular hypercellularity was noted in some areas [Fig 9]. Based on these features, the tumor was diagnosed as low grade fibromyxoid sarcoma. Immunohistochemistry was performed to confirm the diagnosis. The neoplasm stained strongly and diffusely for vimentin [Fig 10]. Staining for other markers including CD 34, CD 68, smooth muscle actin and S 100 were negative [Fig 11].

Bottom Line: Very few cases of intra-abdominal location have been reported.We report a 37 year old man who presented with an abdominal mass and dragging pain.Pre-operative imaging suggested the possibility of a subcapsular hemangioma of liver.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgical Oncology, MS Ramaiah Medical College, Bangalore, India. drkhari@yahoo.com

ABSTRACT

Background: Low grade fibromyxoid sarcomas (LGFMS) are very rarely seen. They commonly arise from deep soft tissues of the lower extremities. Very few cases of intra-abdominal location have been reported.

Case presentation: We report a 37 year old man who presented with an abdominal mass and dragging pain. Pre-operative imaging suggested the possibility of a subcapsular hemangioma of liver.

Conclusions: Laparoscopy was useful to locate the tumor as arising from falciform ligament and made the subsequent surgery simpler. This is one of the large fibromyxoid sarcomas to be reported.

Show MeSH
Related in: MedlinePlus