Sarcomas of the sellar region are uncommon and unexpected tumors. Here, we review the cases reported in literature via a systematic search.

Ninety-four patients, 58.5% male with mean age of 39.2 ± 17.2 years were included. Fifty-seven (62%) had soft tissue sarcomas (STS) and 35 (38%) bone sarcomas (BS). Sarcoma was a primary tumor in 66%, developed after radiotherapy in 31.9% and 7.4% were metastatic. Median time between radiotherapy and sarcoma development was 10.5 (11) years. Main presentation symptoms were visual disorders (87.9%), headache (61.5%) and III cranial nerve palsy (24.1%). After surgery, sarcoma persisted or recurred in 82.3% and overall mortality reported was 44.6% with 6.5 (14) months of median survival. Tumor appeared earlier in BS compared to STS (34.4 ± 15.1 vs. 42.6 ± 17.6 years), p = 0.034 and complete tumor resection was achieved more often (41.3% vs. 4.4%), p =  < 0.001. Condrosarcoma and rhabdomyosarcoma were more frequent subtypes among primary tumors while fibrosarcoma was among post-radiation sarcomas. Tumor size was larger in radiation associated sarcomas (mean maximum diameter 46.3 ± 9.3 vs. 29.1 ± 8.0 mm, p = 0.004) and persistency/recurrence was similar in both groups (70.1 vs. 73.3%, p = 0.259).

Sarcomas appear as mass effect symptoms in the middle aged population, mainly as primary tumors, but one third is associated with radiotherapy. Surgery is commonly not curative, mortality rate is high and death ensues shortly after diagnosis.

Soft tissue sarcoma (STS) of the sella is exceptionally rare. We conducted a case series, literature review, and nationwide analysis of primary and iatrogenic (radiation-associated) STS of the sella to define the clinical course of this entity.

This study employed a multi-institutional retrospective case review, literature review, and nationwide analysis using the National Cancer Database (NCDB).

We report five patients who were diagnosed at three institutions with malignant STS of the sella. All patients presented with symptoms related to mass effect in the sellar region. All tumors extended to the suprasellar space, with the majority displaying extension into the cavernous sinus. All patients underwent an operation via a transsphenoidal approach with a goal of maximal safe tumor resection in four patients and biopsy for 1 patient. Histopathologic evaluation demonstrated STS in all patients. Post-operative adjuvant radiotherapy and chemotherapy were given to 2 and 1 out of 4 patients with known post-operative clinical course, respectively. The 1-year and 5-year overall survival rates were 100% (5/5) and 25% (1/4). Twenty-two additional reports of primary, non-iatrogenic STS of the sella were identified in the literature. Including the three cases from our series, treatment included resection in all cases, and adjuvant radiotherapy and chemotherapy were utilized in 50% (12/24) and 17% (4/24) of cases, respectively. The national prevalence of malignant STS is estimated to be 0.01% among all pituitary and sellar tumors within the NCDB.

We report the prevalence and survival rates of STS of the sella. Multimodal therapy, including maximal safe resection, chemotherapy, and radiotherapy are necessary to optimize outcomes for this uncommon pathology.

Skull base chordomas account for less than 0.2% and chondrosarcomas for less than 0.15% of all intracranial tumors. Although their clinical and imaging presentations are similar, they derive from different origins. Chordomas arise from embryonic remnants of the primitive notochord and chondrosarcomas from primitive mesenchymal cells or from the embryonic rest of the cranial cartilaginous matrix. Both entities are characterized by infiltration and destruction of the surrounding bone and soft tissue and a high locoregional recurrence rate. Chondrosarcomas, when treated with similar complex strategies, display a much better prognosis than chordomas. The overall survival is approximately 65% for chordomas and 80% for chondrosarcomas at 5 years and 30 and 50%, respectively, at 10 years. Chordomas are divided into the following 3 histological types: classical (conventional), chondroid, and dedifferentiated. Chondrosarcomas have conventional, mesenchymal, clear cell, and dedifferentiated subgroups. Both tumor entities often present with nonspecific symptoms, and headaches are the most reported initial symptom. Computed tomography and magnetic resonance imaging are required to determine the tumor localization and the extent of tumor growth. The treatment philosophy is to maximize tumor resection, minimize morbidity, and preserve function. Neurosurgical approaches commonly used for the resection of intracranial chordomas and chondrosarcomas are transsphenoidal, transbasal, cranio-orbitozygomatic, transzygomatic extended middle fossa, transcondylar, and transmaxillary approaches. Chordomas and chondrosarcomas are not sensitive to chemotherapy and there are no approved drugs for their treatment. The present treatment concept is a combination of surgical resection with a maximal excision and preserving patients' quality of life by adjuvant radiotherapy for both chordomas and chondrosarcomas.

Low-grade chondrosarcoma (LGC) is a very rare intracranial tumor, particularly in the sellar area. Herein, we describe an unusual case of LGC occurring in the sellar area. A 52-year-old man presented with diminution of vision for more than 3 months, but did not exhibit headaches reported in previous cases. MRI showed that the maximum size of the tumor was 7 cm on the left side of the saddle. We characterized the specific pathological characteristics. Histologically, the tumor had polypoid areas and a lobulated growth pattern under low-power examination. At high magnification, the tumor consisted of small cells with hyperchromatic nuclei in the cartilage matrix, with an alternating loose hypocellular zone and rich myxoid area. In our case, LGC needed to be distinguished from chordoma. Immunohistochemically, the tumor cells showed diffuse positivity for S-100 and vimentin, IDH1 was weakly cytoplasm positive. The Ki-67 labeling index was less than 5%. Additionally, AE1/3, EMA, and CK19 were negative, which could be used to exclude chordoma. This case report expands the literature on LGC and will help clinicians and pathologists better understand this entity.

Chondrosarcoma is a malignant tumor that originates from mesenchymal cells that have differentiated into chondrocytes, often growing laterally, rarely seen in the cranium, and seldom seen in the saddle area. We believe that only a few cases have been reported in the literature. We report a case of pituitary fossa chondrosarcoma, which was completely resected by an extended endoscopic endonasal approach, and a literature review.

A 20-year-old man was admitted to hospital with bilateral temporal headache and blurred vision, without any history of sexual dysfunction or diabetes insipidus. Endocrine function was normal. Computed tomography of the head showed calcified sellar lesions and sellar bone destruction, which were closely associated with the right cavernous sinus. Magnetic resonance imaging showed saddle area space-occupying lesions, with low signal on the T1-weighted image and high signal on the T2-weighted image, uneven enhancement by enhanced scanning, and unclear pituitary display. The tumor was completely resected by an extended endoscopic endonasal approach and confirmed by magnetic resonance imaging. Postoperative pathology revealed conventional chondrosarcoma (World Health Organization grade II). Postsurgical visual acuity also improved. The patient did not receive radiotherapy or chemotherapy. No recurrence was found at 10-month follow-up.

Sellar region chondrosarcoma is rare. For space-occupying lesions in this area, chondrosarcoma should be considered and not necessarily pituitary adenoma, craniopharyngioma, meningioma, and chordoma. The extended endoscopic endonasal approach represents a good treatment option for sellar area chondrosarcoma.