Malignant Granular Cell Tumor of the Esophagus: A Case Report.

INTRODUCTION: Malignant granular cell tumor (MGCT) of the esophagus is an extremely rare malignancy with a poor prognosis. Literature describing this condition is not sufficient, especially regarding long-term survival. PRESENTATION OF CASE: A 52-year-old woman presented with dyspnea and slow onset dysphagia. The endoscopy, endoscopic ultrasound (EUS), bronchoscopy, and positron emission tomography (PET)/computed tomography (CT) supported the suspicion of esophageal gastrointestinal stromal tumor (GIST). Open wedge esophagectomy and tracheal resection were performed. The histology proved periodic acid-Schiff (PAS)-positive granules in epithelial cells, hyperchromatic nuclei and the positivity of Protein soluble in 100% ammonium sulfate (S-100), vimentin, neuron-specific enolase, laminin, and myelinic proteins. Local recurrence after 10 months required a two-phase esophagectomy with retrosternal gastroplasty. Bone, liver, and mediastinal metastases occurred 6 months later, with overall survival of 34 months. DISCUSSION: Preoperative histological confirmation is often not reliable. Tracheal invasion increases the perioperative risk and the probability of an unsuccessful resection. Esophagectomy or radical R0 local resection is the only known curative therapy. Repeated resections may increase survival in case of locoregional recurrence. Radiotherapy has a potential for palliative care. CONCLUSION: Esophageal MGCT requires a detailed presentation including long-term survival. Early surgical removal of intramural esophageal neoplasms with potentially malignant features is highly recommended. Radical and/or repeated esophageal resections are the only known therapies with curative potential.

Metastatic granular cell tumor to the breast diagnosed by fine needle aspiration cytology: A case report with review of the literature.

Granular cell tumors (GCT) are mesenchymal neoplasms of Schwann cell/neural origin. Malignant granular cell tumors (MGCTs) represent <1-2% of all GCT and defined as tumors demonstrating metastases or destructive local growth. Other clinical parameters suggestive of malignancy include rapid growth, size > 4 cm and necrosis. An apparently inconsistent set of histological features have been described in MGCT. Although the histologic parameters of a GCT are not always predictive of biologic behavior, the presence of atypical features may be indicative of an aggressive clinical behavior (recurrence and metastases). A preoperative estimate of features suggestive of malignancy is important for treatment and prognostication. Diagnosis and prognostication from preoperative fine needle aspiration (FNA) cytology is hampered by the fact that only a few case reports on cytologic features of malignant GCT have been published. We report a case of metastatic MGCT to breast and compare cytologic features to that of primary breast GCT and apocrine/histiocytoid variants of breast carcinoma.

The predictive accuracy of sentinel nodes mapping in the setting of pulmonary metastasectomy.

This is the first study to evaluate the feasibility of mediastinal lymph node dissection (MLND) based on sentinel lymph node (SLN) status during pulmonary metastasectomy. A total of 22 patients (16 men, 6 women; age 63.3 ± 7.01 years) who were candidates for metastasectomy through segmentectomy or lobectomy with MLND owing to cancers metastatic to the lung were enrolled in this study. Radiotracer was administered at the peritumoral region before surgery or soon after initiating surgery. During the operation, the radioactivity of the lymph nodes (ex vivo) was counted with a handheld gamma probe after MLND. Lobectomy was performed in 17 patients, and segmentectomy, in 5 patients. The number of dissected lymph nodes per patient was 14.4 ± 8.69 (range, 5-36). In all patients, the SLN could be detected, and the number of SLNs identified was 2.0 ± 1.15 (range, 1-5) per patient. Lymph node metastasis was identified in 3 of the 22 patients (13.6%), and none of the 3 patients with N1 or N2 disease had false-negative SLNs. SLN identification might be an indicator of whether or not MLND should be performed during pulmonary metastasectomy. However, further large-volume and multi-institutional studies are needed.

Primary cutaneous malignant granular cell tumor: an immunohistochemical study and review of the literature.

Granular cell tumors (GCTs) are uncommon soft tissue tumors characterized by cytoplasmic granular appearance of the neoplastic cells. Malignant granular cell tumors (MGCTs) comprise less than 2% of GCTs and are mostly found in the subcutaneous soft tissues of the lower extremities, especially the thighs. Very few cases have been reported in the skin. The uncommon occurrence of cutaneous MGCTs and their histopathologic similarities with their benign counterpart make difficult the diagnosis of this particular malignancy. We describe a primary cutaneous MGCT that presented as a left posterior chest wall mass in a 51-year-old woman. Local excision was performed for the primary tumor, which was first interpreted as an atypical GCT, but 3 months later a left axillary mass appeared, and subsequent axillary lymph node dissection demonstrated metastatic disease in 4 of 12 excised lymph nodes. We report the immunophenotype of this primary cutaneous MGCT, which was studied with an ample panel of antibodies and compare our results with those of the few previously reported cases in the skin and subcutaneous soft tissues.

A case of metastatic non-neural granular cell tumor in a 13-year-old girl.

Conventional granular cell tumor represents a mesenchymal neoplasm observed in a variety of locations and is now believed to be of Schwann cell origin. Granular cell change has also been observed in a variety of different tumors, but recently described in the skin has been a distinct entity termed non-neural granular cell tumor, which lacks expression of S100 protein and is of uncertain histogenesis. This tumor typically displays a greater degree of nuclear atypia and mitotic activity than conventional granular cell tumor but appears to behave in a relatively benign fashion, as only two previous instances of lymph node metastasis have been documented. Herein, we report a case of non-neural granular cell tumor arising on the back of a 13-year-old girl, and later axillary lymph node metastasis with extracapsular extension was observed.

Granular cell tumor on perianal region: a case report.

Granular cell tumor (GCT) was first described by Abrikossoff in 1926. GCT is a rarely seen soft tissue tumor and is generally benign. While the tumor can be seen in all parts of the body it is generally located on the head and neck region, and especially on the tongue. GCT is rarely seen in the anal-perianal region. In accordance with literature this case was reported because it was thought to be the 27th anal-perianal located GCT case. In this case report, approximately 0,5-1 cm pedunculated polypoid lesion was determined in the perianal region during the physical examination of a 23 year old female patient who applied with palpable mass complaint in the perianal region. Lesion in the patient was totally excited with healthy skin-subcutaneous tissue under local anesthesia. A benign granular cell tumor was detected in the histopathological examination. Positive staining was monitored immunohistochemically with S-100 and neuron specific enolase (NSE). GCT is a rarely seen tumor in the anal-perianal region and its malign transformation rate is very low. Even lesions seen in the perianal region have clinically a benign appearance, a histopathological examination should be conducted and also GCT should be kept in mind during diagnosis. Malign-benign separation of these lesions is difficult so histopathological examination should be conducted with great care. Large local excision in the treatment provides curative treatment. But for those presenting malign transformation further examination must be performed for metastasis. After the treatment local recurrence and metastasis should be considered carefully. Prognosis of metastatic disease is very bad.

Primitive non-neural granular cell tumour with lymph node metastasis.

Primitive non-neural granular cell tumour (PNGCT) is a rare tumour of uncertain lineage. This report describes a case of PGNCT with lymph node metastasis. This is thought to be only the second reported case with lymph node involvement.

[Pulmonary metastases from Abrikossoff's tumour. Transformation capability of a benign granular cell tumour]. / Métastases pulmonaires d'une tumeur d'Abrikossoff. Transformation ou potentiel évolutif d'une tumeur bénigne à cellules granuleuses.

INTRODUCTION: Abrikossoff's tumour or granular cell tumour is usually benign involving multiple anatomical sites, most frequently the head, neck and airways. Occasional observations of aggressive malignant tumours have been reported, associated with a poor prognosis. CASE REPORT: We report the case of a mammary Abrikossoff's tumour, initially considered benign and treated solely by local surgery. Seven years later the tumour was responsible for the development of sub-cutaneous and pulmonary metastases. Local surgery was again the only treatment given in the absence of evidence for the effectiveness of alternative treatment with chemotherapy or radiotherapy. CONCLUSION: This original observation reports the case of a benign granular cell tumour that underwent malignant transformation after an interval of 7 years as indicated by the clinical progress and the cellular proliferation index Ki-67.

Heart transplantation treatment for a malignant cardiac granular cell tumor: 33 months of survival.

A malignant granular cell tumor is a high-grade sarcoma with a high rate of metastasis, local recurrence and short survival. We describe a patient who underwent orthotopic heart transplantation due to a metastatic unresectable malignant granular cell tumor. At thirty-three months following this procedure she is alive and free of recurrence.

Malignant granular cell tumor in larynx mimicking laryngeal carcinoma.

A 72-year-old man presented to our clinic with progressed husky voice, dysphagia and globus pharyngeus. Fiberoptic laryngoscopy showed a large subglottic mass with an irregular surface. A chest roentgenogram revealed multiple nodules over the right upper and lower lobes. Under the impression of malignant laryngeal tumor with lung metastasis, he underwent direct laryngeal biopsy and excision. Pathologic findings showed malignant granular cell tumor. Postoperative palliative chemotherapy was done for his lung metastasis. The multiple pulmonary nodules were decreased in size and number but not complete remission. The laryngeal tumor has not recurred after a 14-month follow-up.

[Pulmonary and pleural metastasis of a malignant granular cell tumor]. / Pulmonale und pleurale Metastasierung durch einen malignen Granularzelltumor.

HISTORY AND CLINICAL FINDINGS: A 72-year-old woman, who suffered from increasing dyspnea and productive cough was admitted to hospital. Clinical examination revealed a reduced respiratory sounds over the left lung and a painless and unmovable tumor in the area of the left hip. CLINICAL AND LABORATORY TESTS: Apart from hypoxemia (pO2 7.81 kPa) the laboratory values did not indicate any pathological findings. The X-ray and CT-scan of the chest showed a few spotty shadows and pleura effusion on the left. No tumor cells were detected in the pleural effusion. In biopsies of the visceral and parietal pleura as well as biopsy within the tumor in the area of the left hip there were clusters of tumor cells of a granular cell tumor. DIAGNOSIS, TREATMENT AND CLINICAL COURSE: Because the same tumor cell type was detected in the visceral and parietal pleura and wihtin the tumor at the left gluteus area, we diagnosed a malignant granular cell tumor. The CT-scan was suspicious of lung metastasis. The primary tumor was located in the area of left hip. The patient was in a poor general condition and she suffered from an extensive metastastic disease; curative treatment was not possible. A pleurodesis was performed. The patient died nine months after initial diagnosis. CONCLUSION: A rare malignant granular cell tumor was discovered by detecting tumor tissue of granular cell tumor in the pleura and within a tumor in the left gluteus area. Another indication of a metastatic disease were multifocal lesions in the lung detected by CT-scan. Curative treatment was not possible.

Diagnosis of malignant granular cell tumor by fine needle aspiration cytology:.

BACKGROUND: Malignant granular cell tumors (GCT) are the rarest of all sarcomas, and the histologic differentiation from their benign counterpart may be extremely difficult or impossible unless metastatic disease is demonstrated. To our knowledge, this is the first report of a malignant GCT diagnosed by fine needle aspiration (FNA) cytology. CASE: A 70-year-old, Caucasian female presented with a progressively enlarging left supraclavicular mass. FNA of the mass revealed a metastatic tumor cytologically consistent with GCT. With this diagnosis, a search for other metastatic sites was initiated. Computed tomography (CT) scan revealed several tumor nodules in the lungs and liver. CT-guided FNA and tru-cut needle biopsy of a liver mass confirmed the diagnosis of metastatic GCT. In searching for a primary site, the patient revealed a clinical history of having had a tumor removed from her back two months before; it was reported to be an atypical GCT. Comparison of the three tumors revealed similar histologic, cytologic and immunohistochemical features. CONCLUSION: Evidence of mild to moderate cytologic atypia; increased mitotic activity; locally aggressive growth; increased proliferative activity as demonstrated by immunohistochemical evaluation of proliferation markers; and DNA ploidy analysis, as reported for this case, may be helpful in predicting malignant behavior of GCTs.