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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Case of the Month
Case of the Month
Clinical Profile:
Clinical Profile:
A 55 year old man presented with constipation and vague pelvic pain since two weeks.
A 55 year old man presented with constipation and vague pelvic pain since two weeks.
He also complained of loss of weight since one month.
He also complained of loss of weight since one month.
There was no history of similar complaints in the past..
There was no history of similar complaints in the past..
Radiological findings:
Radiological findings:
Fig. 1
Fig. 1
Fig. 2
Fig. 2
Radiograph of the pelvis in AP and lateral view reveals an ill defined soft tissue opacity in the pelvis with no lucencies or calcification within. Both pelvic bones and proximal femur are normal (Fig. 1)
Radiograph of the pelvis in AP and lateral view reveals an ill defined soft tissue opacity in the pelvis with no lucencies or calcification within. Both pelvic bones and proximal femur are normal (Fig. 1)
On CT examination with rectal contrast (Fig. 2), there is a large heterogeneous hyperdense mass lesion measuring 10x8x12cm seen in the perirectal region, posterior to the upper mid rectum and displacing the rectum anteriorly. There is no proximal bowel dilation. Few tiny specks of calcifications are seen within the lesion.
On CT examination with rectal contrast (Fig. 2), there is a large heterogeneous hyperdense mass lesion measuring 10x8x12cm seen in the perirectal region, posterior to the upper mid rectum and displacing the rectum anteriorly. There is no proximal bowel dilation. Few tiny specks of calcifications are seen within the lesion.
No erosions or focal lesion seen in the sacrum.
No erosions or focal lesion seen in the sacrum.
Fig. 3 a
Fig. 3 a
Fig. 3 b
Fig. 3 b
Multiplanar Multiecho MRI (Fig. 3a,3b) of pelvis with contrast reveals a large heterogeneously enhancing, T2 heterogeneously hyperintense presacral mass lesion measuring 10x8x12cm (APxTRAxCC), displacing the upper-mid rectum anteriorly. It shows central T2 hyperintense non enhancing component, in keeping with necrosis.
Multiplanar Multiecho MRI (Fig. 3a,3b) of pelvis with contrast reveals a large heterogeneously enhancing, T2 heterogeneously hyperintense presacral mass lesion measuring 10x8x12cm (APxTRAxCC), displacing the upper-mid rectum anteriorly. It shows central T2 hyperintense non enhancing component, in keeping with necrosis.
Few tortuous flow voids are seen within the lesion.
Few tortuous flow voids are seen within the lesion.
The peripheral heterogeneously enhancing solid component shows diffusion restriction on DWI with corresponding hypointensity on ADC.
The peripheral heterogeneously enhancing solid component shows diffusion restriction on DWI with corresponding hypointensity on ADC.
The fat planes of the lesion with upper mid rectum are effaced whereas the fat planes with the sacrum are maintained.
The fat planes of the lesion with upper mid rectum are effaced whereas the fat planes with the sacrum are maintained.
Treatment:
Treatment:
Midline incision was taken in the lower abdomen, presacral mass was identified, multiple arterial feeders were identified from Inferior mesenteric artery and were ligated. As it was difficult to mobilise the lesion from the surrounding planes another incision was taken from the perineum.
Midline incision was taken in the lower abdomen, presacral mass was identified, multiple arterial feeders were identified from Inferior mesenteric artery and were ligated. As it was difficult to mobilise the lesion from the surrounding planes another incision was taken from the perineum.
The lesion (Fig. 5) was stuck to the posterior wall of upper mid rectum, however the planes with the sacrum were maintained. The lesion was excised along with 8cm of upper mid rectum and coccyx while proximal sigmoid colon stoma made . A small anal stump was left for anastomosis.
The lesion (Fig. 5) was stuck to the posterior wall of upper mid rectum, however the planes with the sacrum were maintained. The lesion was excised along with 8cm of upper mid rectum and coccyx while proximal sigmoid colon stoma made . A small anal stump was left for anastomosis.
During mobilisation of the lesion from its surrounding planes, the presacral venous plexus was injured resulting in loss of approximately 450-500cc of blood.
During mobilisation of the lesion from its surrounding planes, the presacral venous plexus was injured resulting in loss of approximately 450-500cc of blood.
Fig. 4
Fig. 4
Patient developed pelvic collection of approximately 70cc in the postoperative 5th day for which Vaccum Assisted drainage was done. The patient is currently recovering in the surgical ward.
Patient developed pelvic collection of approximately 70cc in the postoperative 5th day for which Vaccum Assisted drainage was done. The patient is currently recovering in the surgical ward.
Pathological diagnosis:
Pathological diagnosis:
Excision biopsy reveals (Fig, 5)
Excision biopsy reveals (Fig, 5)
•Section shows high grade tumour mainly composed of spindle cells arranged in various patterns like sheets, cords and storiform pattern. The cells are pleomorphic, with nuclear atypia. Stroma is predominantly fibro collagenous. Mitotic figures and necrosis are also seen.
•Section shows high grade tumour mainly composed of spindle cells arranged in various patterns like sheets, cords and storiform pattern. The cells are pleomorphic, with nuclear atypia. Stroma is predominantly fibro collagenous. Mitotic figures and necrosis are also seen.
•On immunohistochemistry the tumour cells are positive for CD-34, weakly positive for SMA, negative for S-100 and synaptophysin.
•On immunohistochemistry the tumour cells are positive for CD-34, weakly positive for SMA, negative for S-100 and synaptophysin.
Fig. 5
Fig. 5
Final diagnosis:
Final diagnosis:
Presacral Solitary Fibrous Tumour.
Presacral Solitary Fibrous Tumour.
Discussion:
Discussion:
Solitary fibrous tumors (SFT) are a rare neoplasm of mesenchymal origin that comprise less than 2% of all soft tissue tumors. The majority are benign, although up to 20% may be malignant.
Solitary fibrous tumors (SFT) are a rare neoplasm of mesenchymal origin that comprise less than 2% of all soft tissue tumors. The majority are benign, although up to 20% may be malignant.
Clinical presentation:
Clinical presentation:
Although typically asymptomatic, it may present with symptoms and signs related to extrinsic compression of adjacent organs. Rarely solitary fibrous tumors result in non-islet cell tumor hypoglycemia (NICTH)
Although typically asymptomatic, it may present with symptoms and signs related to extrinsic compression of adjacent organs. Rarely solitary fibrous tumors result in non-islet cell tumor hypoglycemia (NICTH)
•Pathology
•Pathology
•Location
•Location
•The majority of solitary fibrous tumors occur in an intrathoracic location but up to one third may occur in extrathoracic locations, and as such they may be encountered essentially anywhere, including :
•The majority of solitary fibrous tumors occur in an intrathoracic location but up to one third may occur in extrathoracic locations, and as such they may be encountered essentially anywhere, including :
•Pleura
•Pleura
•Spinal cord
•Spinal cord
•Dura
•Dura
•Head and neck
•Head and neck
•Extremities
•Extremities
•Abdominal parenchymal organs
•Abdominal parenchymal organs
•Retroperitoneum
•Retroperitoneum
•Peritoneum
•Peritoneum
•Pelvic organs
•Pelvic organs
•Treatment and prognosis
•Treatment and prognosis
•benign SFT <10 cm in size typically has a favourable outcome with surgical resection alone
•benign SFT <10 cm in size typically has a favourable outcome with surgical resection alone
•SFT >10 cm that also harbors malignant foci, and has positive surgical margins tends to have a poor prognosis despite surgical treatment
•SFT >10 cm that also harbors malignant foci, and has positive surgical margins tends to have a poor prognosis despite surgical treatment
•optimal adjuvant therapy for this group is unknown, and close-interval follow-up is advised because there is an increased incidence of local recurrence
•optimal adjuvant therapy for this group is unknown, and close-interval follow-up is advised because there is an increased incidence of local recurrence
References
References
•1. Vallat-decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am. J. Surg. Pathol. 1998;22 (12): 1501-11. Am. J. Surg. Pathol. (link) - Pubmed citation
•1. Vallat-decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am. J. Surg. Pathol. 1998;22 (12): 1501-11. Am. J. Surg. Pathol. (link) - Pubmed citation
•2. Ogungbo B, Prakash S, Kulkarni G et-al. Cervical intra-/extramedullary solitary fibrous tumour. Br J Neurosurg. 2005;19 (3): 254-7. doi:10.1080/02688690500208528 - Pubmed citation
•2. Ogungbo B, Prakash S, Kulkarni G et-al. Cervical intra-/extramedullary solitary fibrous tumour. Br J Neurosurg. 2005;19 (3): 254-7. doi:10.1080/02688690500208528 - Pubmed citation
•3. Metellus P, Bouvier C, Guyotat J et-al. Solitary fibrous tumors of the central nervous system: clinicopathological and therapeutic considerations of 18 cases. Neurosurgery. 2007;60 (4): 715-22. doi:10.1227/01.NEU.0000255418.93678.AD - Pubmed citation
•3. Metellus P, Bouvier C, Guyotat J et-al. Solitary fibrous tumors of the central nervous system: clinicopathological and therapeutic considerations of 18 cases. Neurosurgery. 2007;60 (4): 715-22. doi:10.1227/01.NEU.0000255418.93678.AD - Pubmed citation
•4. Suter M, Gebhard S, Boumghar M et-al. Localized fibrous tumours of the pleura: 15 new cases and review of the literature. Eur J Cardiothorac Surg. 1998;14 (5): 453-9. Eur J Cardiothorac Surg (link) - Pubmed citation
•4. Suter M, Gebhard S, Boumghar M et-al. Localized fibrous tumours of the pleura: 15 new cases and review of the literature. Eur J Cardiothorac Surg. 1998;14 (5): 453-9. Eur J Cardiothorac Surg (link) - Pubmed citation
•5. Lee KS, Im JG, Choe KO et-al. CT findings in benign fibrous mesothelioma of the pleura: pathologic correlation in nine patients. AJR Am J Roentgenol. 1992;158 (5): 983-6. AJR Am J Roentgenol (abstract) - Pubmed citation
•5. Lee KS, Im JG, Choe KO et-al. CT findings in benign fibrous mesothelioma of the pleura: pathologic correlation in nine patients. AJR Am J Roentgenol. 1992;158 (5): 983-6. AJR Am J Roentgenol (abstract) - Pubmed citation
•7. Haaga JR, Boll D. CT and MRI of the whole body. Mosby. (2009) ISBN:0323053750. Read it at Google Books -
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•8. Robbins SL, Kumar V, Abbas AK et-al. Robbins and Cotran Pathologic Basis of Disease. W.B. Saunders Company. (2010) ISBN:1416031219. Read it at Google Books -
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•9. Dähnert WF. Radiology Review Manual. Lippincott Williams & Wilkins. (2011) ISBN:1609139437.
•9. Dähnert WF. Radiology Review Manual. Lippincott Williams & Wilkins. (2011) ISBN:1609139437.
•10. Gengler C, Guillou L. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. Histopathology. 2006;48 (1): 63-74. doi:10.1111/j.1365-2559.2005.02290.x - Pubmed citation
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•11. Khowaja A, Johnson-Rabbett B, Bantle J et-al. Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor-2 from a malignant renal solitary fibrous tumor - clinical case and literature review. BMC Endocr Disord.14 (1): 49. doi:10.1186/1472-6823-14-49 - Free text at pubmed - Pubmed citation
•11. Khowaja A, Johnson-Rabbett B, Bantle J et-al. Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor-2 from a malignant renal solitary fibrous tumor - clinical case and literature review. BMC Endocr Disord.14 (1): 49. doi:10.1186/1472-6823-14-49 - Free text at pubmed - Pubmed citation
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