A Case of Symptomatic Granular Cell Tumor of the Pituitary Treated with Stereotactic Radiosurgery.

BACKGROUND: Granular cell tumors (GCT) of the pituitary are rare. Surgery is considered the primary management option. However, complete resection is often difficult, and surgery is associated with high rates of postoperative complications. AIMS: To report a unique case of pituitary GCT treated with stereotactic radiosurgery (SRS). CASE DESCRIPTION: We report a case of a 40-year-old female with endocrine dysfunction who underwent stereotactic biopsy and then SRS for the management of a suprasellar granular cell tumor. Over the ensuing 10 years, tumor regression was observed. Thirteen years after SRS, the patient remained asymptomatic; however, follow-up MRI demonstrated tumor progression. Fifteen years after SRS, the patient required endoscopic endonasal surgery after developing a new optic neuropathy. Her images demonstrated further tumor growth beyond the targeted area. CONCLUSION: SRS resulted in long-term tumor control without additional endocrine dysfunction, but the onset of new optic neuropathy associated with delayed tumor growth prompted surgical decompression.

Adjuvant radiation therapy for malignant Abrikossoff's tumor: a case report about a femoral triangle localisation.

BACKGROUND: Granular cell or Abrikossoff's tumors are usually benign however rare malignant forms concern 1 to 3% of cases reported. Pelvic locations are exceptional. CASE PRESENTATION: We report a case of a 43-years-old patient who had a benign Abrikossoff's tumor localized in the right femoral triangle diagnosed at the biopsy. The patient underwent a surgical tumorectomy and inguinal lymph nodes resection. Histologically, the tumor showed enough criteria to give diagnosis of malignancy: nuclear pleomorphism, tumor cell spindling, vesicular nuclei with large nucleoli. Moreover, five lymph nodes were metastatic. Immunohistochemistry findings confirmed the diagnosis of granular cell tumor which is positive for S100 protein and CD68 antibodies. The mitotic index was nevertheless low with a Ki67 labeling index of 1-2%. A large surgical revision with an inguinal curage following radiotherapy were decided on oncology committee. Adjuvant radiotherapy on the tumor bed and right inguinal area of ​​50 Gy in conventional fractionation was delivered with the aim of reducing local recurrence risk. There was no recurrence on longer follow-up (10 months post radiotherapy). CONCLUSIONS: Adjuvant radiotherapy seems an appropriate therapeutic approach, even if controversial, given that some authors report effectiveness on local disease progression.

Atypical granular cell tumor-apropos of a case with indeterminate malignant potential.

Malignant granular cell tumor is relatively uncommon, constituting 1%-2% of all granular cell tumors.We describe a case of a 53-year-old woman who presented with a 4.5 X 5.5-cm nodule on her left lower abdomen. An initial punch biopsy revealed a pandermal infiltrate of epithelioid cells with finely granular cytoplasm and vesicular nuclei-histologic features consistent with a granular cell tumor. Given the large size of the neoplasm, an excisional biopsy was recommended that revealed in addition to the above, focal ulceration, epithelioid cells with vesicular nuclei and prominent nucleoli, increased mitotic activity, pleomorphism, and epidermotropism with pagetoid spread-features that were histologically concerning for malignant transformation. We present this case to increase awareness of an uncommon entity and to emphasize the pitfalls associated with incomplete removal and an inadequate biopsy.

Symptomatic granular cell tumour of the pituitary.

Granular cell tumours (GCT) and pituicytomas are rare tumours seen in the sellar suprasellar region which are believed to arise from either the infundibulum or the posterior lobe of the pituitary gland. Whether they are two distinct lesions or represent one single pathological entity remains a matter of debate. We report the case of a 42-year-old male with a granular cell neoplasm of the sellar suprasellar region and attempt to discuss the controversies shrouding this distinct clinicopathological entity.

Unusual presentation of a malignant granular cell tumor of the pelvis: case report and literature review.

BACKGROUND: Malignant granular cell tumors are among the rarest of soft tissue cancers, currently understood to be of Schwann cell origin. As with their benign counterparts, malignant granular cell tumors (MGCTs) have a wide anatomic distribution and carry a poor prognosis, with recurrence and metastasis typically within 1 year of diagnosis. Only a handful of MGCTs have been described in the pelvis. CASE: We describe a case of malignant granular cell tumor that presented as a pararectal mass associated with severe rectal pain. The patient underwent pelvic exenteration and postoperative radiation therapy. She recurred with evidence of liver metastases on imaging studies 8 months following her exenteration. CONCLUSION: We discuss the diagnosis and prognosis of malignant granular cell tumors arising in the pelvis.

Yttrium-aluminum-garnet laser therapy of esophageal granular cell tumor.

BACKGROUND: Granular cell tumor (GCT) is a rare lesion. Approximately 4% to 6% of these tumors occur in the gastrointestinal tract, one-third of them affecting the esophagus. Almost all GCTs are benign lesions. Approximately 1% to 3% are malignant. Endoscopic ultrasonography (EUS) is a diagnostic support. The best treatment for esophageal GCT is not yet clear, whether surgical excision, periodic observation, endoscopic excision, or yttrium-aluminum-garnet (YAG) laser therapy. METHODS: From November 1992 to December 2000, four patients with GCTs of the esophagus were observed. All the patients underwent EUS evaluation and endoscopic YAG laser therapy of the esophageal neoplasm. At each session, a biopsy at the tumor site was obtained. The treatment was continued until endoscopic and histologic evidence of the tumor disappeared. RESULTS: After the YAG laser therapy, no evidence of the tumor was found in any of the four patients with esophageal GCT. At this writing, the patients remain disease free after a mean follow-up period of 66 months. No complication has been observed. Only four sessions for each patient were necessary to eliminate the tumor. CONCLUSIONS: Therapy with YAG laser was effective in all four patients with esophageal GCT, and complete necrosis of the submucosal neoplastic cells was achieved. Endoscopic YAG laser therapy appears to be a good compromise between esophageal dissection and long-term observation without tumor excision. Esophageal laser therapy is safe if correctly used, and previous EUS evaluation increases treatment safety.

Malignant bronchial Abrikossoff's tumor and small cell lung cancer: a case report and review.

A 61-year-old male Caucasian smoker patient underwent chest radiography and CT scan for persistent non-inflammatory cough, which showed a left bronchial unresectable mass. Bronchoscopy showed an endobronchial mass; washing cytology was negative and histology findings suggested diagnosis of granular cell tumor (GCT), also called Abrikossoff's tumor. After 3 weeks a new washing cytology test revealed the presence of small cell lung cancer (SCLC). A CT-scan and chest radiography showed a 30% increase in the maximum diameter of the lesion, clinically defining the primary neoplasm as malignant. The patient was referred to our institution and started chemotherapy with cisplatin and etoposide. After 6 cycles of treatment, the CT scan showed complete, disappearance of the neoplasm and bronchoscopy examination showed no endobronchial lesion, defining the mucosal surface as normal. We have reviewed and summarized the international literature with regard to bronchial localization of malignant granular cell tumor and its association with SCLC, therefore concluding that our case is the first malignant endobronchial GCT linked to SCLC.

Malignant granular cell tumor metastatic to the orbit.

OBJECTIVE: Malignant granular cell tumor is a rare type of soft tissue sarcoma. To our knowledge, ocular (eyelid) involvement has been described in only two cases. Herein, we report the clinicopathologic features of an unusual case of malignant granular cell tumor metastatic to the orbit. DESIGN: Observational case report. METHODS: Retrospective review of the medical record and the histopathologic and electron microscopic findings and review of the literature. RESULTS: A 72-year-old man with biopsy-proven granular cell tumor in the cervical region was initially seen with proptosis and motility disturbance. A magnetic resonance imaging scan showed a large intraconal mass, and biopsy of the orbital mass revealed granular cell tumor. Histopathologic examination of the primary neck tumor and the orbital mass revealed increased nuclear atypia and pleomorphism in the consecutive lesions. The morphologic impression of granular cell tumor was also supported by the immunohistochemical demonstration of S-100 protein expression and ultrastructural findings typical of granular cell tumor. Six months after the orbital involvement, systemic workup revealed multiple apparent bony and lung metastases. CONCLUSIONS: We report the first malignant granular cell tumor metastatic to the orbit and suggest the inclusion of this tumor in the differential diagnosis of metastatic orbital lesions.