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The original neoplasm, a circumscribed intradural mass attached to the left C-6 nerve root, from the first surgery. a: Low-power image of an H & E–stained section showing a largely spindle cell neoplasm with brown melanin pigmentation distributed throughout the lesion. A psammoma body is seen as a blue well-demarcated structure at the upper center of the image. b: S100 immunohistochemistry demonstrating labeling of nuclei and cytoplasm of the neoplastic cells. The unstained structure in the lower right quadrant of the image is a blood vessel. c: Reticulin stain demonstrating abundant pericellular reticulin deposition, seen as a black threadlike material. This finding is highly characteristic of schwannoma and would not be expected in melanoma. d: Highpower detail of a portion of the field seen in panel A, demonstrating ovoid, elongated nuclei, some with small nucleoli and no mitotic figures. Melanin pigment is visible within the cytoplasm of many of the neoplastic cells. e: Immunohistochemistry for the Ki 67 proliferation marker demonstrating labeling of only a rare nucleus in this field, indicated by the round, nucleus-shaped brown labeling, not to be confused with the dustlike and irregularly aggregated cytoplasmic melanin pigment. Original magnification ×100 (A); ×400 (B–E).
Source publication
The authors present a case of intraspinal malignant psammomatous melanotic schwannoma (PMS) not associated with Carney complex and review all reported cases not associated with this syndrome. The focus of this review paper is on the characteristics of the malignant progression of PMS.
A 54-year-old man had a history of squamous cell carcinoma of th...
Contexts in source publication
Context 1
... intradural well-circumscribed tumor showed heavy melanin pigmentation in both the intracytoplasmic and ex- tracellular regions. The neoplasm was composed of spindle and epithelioid cells ( Fig. 2) with atypical nuclei that pos- sessed prominent nucleoli and scattered intranuclear cy- toplasmic inclusions. Immunohistochemical analyses for S100, HMB-45/Melan-A cocktail, and Ki 67 were done. The neoplasm showed extensive cytoplasmic and some nu- clear labeling of S100 (Fig. 2). HMB-45/Melan-A and Ki 67 were negative. A BRAF ...
Context 2
... The neoplasm was composed of spindle and epithelioid cells ( Fig. 2) with atypical nuclei that pos- sessed prominent nucleoli and scattered intranuclear cy- toplasmic inclusions. Immunohistochemical analyses for S100, HMB-45/Melan-A cocktail, and Ki 67 were done. The neoplasm showed extensive cytoplasmic and some nu- clear labeling of S100 (Fig. 2). HMB-45/Melan-A and Ki 67 were negative. A BRAF mutation study was done. How- ever, the assay-specific requirement to generate a result was not met, which could have been due to either nucleic acid concentration below the detectable limits or factors that influenced the integrity of the ...
Context 3
... metastasis of PMS: malignant transformation of the tumor or spreading via CSF after violation of subarach- noid space. It has been previously reported that melanotic schwannoma, regardless of the presence of psammoma bodies or Carney complex, has the risk of malignant trans- formation. 13 In our patient, the first pathology came back as benign (Fig. 2), but the second pathology was read as malignant (Fig. 4). Although the previously reported cases did not discuss the method of recurrence and metastasis, our report indicates that the possible malignant transfor- mation should be taken into consideration for patients with PMS not associated with Carney ...
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Citations
... 22 In some series including patients with intradural lesions, malignant PMS developed recurrence of the tumour within 8 months postoperatively, but the patient without malignant tumour was disease free 16 months postoperatively. 23 Only elevated mitotic activity (Z2 mitotic figures/10 HPF) was found to be associated with a higher risk for aggressive behavior and metastasizing in MS, but the lack of it does not corroborate benign course of the disease. 4 The overall mortality of MS is 10%-17% of all cases in different series. ...
First described by Miller in 1932, melanocytic schwannoma (MS) (melanotic schwannoma, pigmented schwannoma) is a rare variation of peripheral nerve sheet tumours with ectodermal origin occurring predominantly in somatic, but also in the autonomic peripheral system with around two hundred cases in the literature. Predominantly benign tumours, MS are still imaging and pathological challenge and can be easily misdiagnosed with more aggressive peripheral nerve tumours.
We report a case of melanocytic schwannoma on L3 sensory rootlet with systematic literature review of nearly 200 cases presented in intracranial, paraspinal region, thoracic, abdominal or pelvic cavities and skin. Two-thirds of cases are part of Carney complex.
We present a case of a 61-year-old male with a 3-month history of low back pain, progressive numbness and stiffness in the right thigh, shin and knee, tibial and peroneal paresis causing gait disturbance and neurological claudication. MRI findings present “sand clock” type intradural extramedullary tumour formation with extension to the L3 rootlet through right L3-L4 foramen, hypointense on T2 and hyperintense on T1. Pathological diagnosis of sporadic type melanocytic schwannoma was made via immunohistological and ultrastructural analysis. Thirteen months after total resection there was clinical and MRI evidence of recurrence of the tumour. Total resection and radiosurgery was performed with a recurrence free period of 14 months.A gold standard for melanocytic schwannoma treatment is gross total surgical resection. Despite being considered benign tumours, MS have a local or metastatic recurrence of around 13%. MRI imaging in most of the cases is insufficient and only exhaustive pathological and immunohistological examination is the key to diagnosis. Need of postoperative radiation therapy is still controversial. For the first time, a criterion for postoperative adjuvant therapy was established.
... [20] Classical malignant histologic features such as nuclear abnormalities and necrosis are considered worrisome for future malignant behavior of MS. [50] e previous reviews of both sporadic and Carney complex-associated MS have reported metastasis rates from 15% [62] to 42%. [61] Our review of only sporadic spinal MS revealed metastases in 32.7% of cases, [56] 57.9% of those including metastases to the lungs. Due to expanding data that show more aggressive behavior than previously thought, Torres-Mora et al. proposed the reclassification of MS to "melanotic schwannian tumor. ...
Background
Melanotic schwannoma (MS) is a rare variant of peripheral nerve sheath tumor. MS commonly arises along the spinal nerve sheath. Patients most often experience pain along the dermatome of the affected nerve root. Symptoms development is usually insidious. About half of MS cases are associated with Carney complex, a multi-neoplastic disorder. The remaining cases arise spontaneously. About 10–44% of these tumors undergo malignant transformation.
Case Description
We describe a case of hemorrhagic MS presenting as acute chest pain mimicking myocardial infarction, a presentation which has not yet been described in the literature. Neurologic examination did not reveal any abnormalities. Myocardial infarction was ruled out in the ER, and a chest CT angiogram was ordered for evaluation of PE or aortic dissection which revealed an intradural extramedullary dumbbell-shaped mass extending through the left vertebral foramen at the level of T8. MRI revealed a heterogenous mass that was hyperintense with T2 and hypointense with T1-weighted imaging. The patient underwent an open laminectomy of the left T8 and T9 vertebrae and gross total resection (GTR) of a hemorrhagic black tumor. Microscopic examination showed fascicles and nests of plump spindle cells with variable intracellular melanin. Immunohistochemistry showed the cells to be positive for S100, SOX10, HMB-45, and MART-1, confirming diagnosis of MS. Two months after the operation, the patient was doing well and is free of recurrence.
Conclusion
GTR is considered the optimal treatment for MS; radiotherapy and chemotherapy may be considered but have not been shown to improve patient outcomes.
... This pattern is rarely found and therefore is an important point leading to the suspicion of MS. Approximately 40%-50% of MS are psammomatous 6,7) ; approximately half of these cases are idiopathic, whereas the rest are associated with Carney complex 7,8) . Carney complex is an autosomal dominant syndrome characterized by spotty skin pigmentation, endocrine tumors, and myxomas of the heart, skin, and breast 6,7) . ...
... Although the standard treatment for PMS is total resection, the rate of recurrence is higher than that in non-PMS even when complete resection is achieved 8) , thus raising the question whether to perform postsurgical radiotherapy and/or chemotherapy. Some studies have suggested that radiotherapy should be administered to patients in whom only partial resection is possible to decrease the likelihood of metastasis 9) . ...
... PMS is present in 50% of patients with Carney complex (CNC), characterized by spotty skin pigmentation, myxomas of the heart, skin, and breast, and endocrine tumors [5][6][7]. PMS is found sporadically in 55% of cases and is usually benign, but the malignant type tends to metastasize [8][9][10]. The female-male ratio of PMS was 1.4:1 while the mean age was 37 years [4]. ...
... We report a case in which we discuss findings that suggest aggressive behavior and report a number of newer molecular studies of PMS such as the test for BRAF V600E, a marker which is typically present in over 90% of melanomas, was not present in the patient's tumor [9,18]. This case of malignant PMS emphasizes the importance of making the correct diagnosis leading to the recognition of CNC and the uncertainty associated with the correct treatment in cases of malignant variety. ...
Background
Sporadic Spinal Psammomatous Malignant Melanotic Nerve Sheath Tumor (SSP-MMNST) is a rare subgroup of peripheral nerve sheath tumors arising along the spine. Only a few reports of SSP-MMNST have been described. In this paper, we review the literature on SSP-MMNST focusing on clinical, and diagnostic features, as well as investigating possible pathogenetic mechanisms to better implement therapeutic strategies. We also report an illustrative case of a young female presenting with cervicobrachial pain due to two SSP-MMNSTs arising from C5-6 right spinal roots.
Case description
We report a case of a 28-year-old woman presenting with right arm weakness and dysesthesia. Clinical examination and neuroimaging were performed, and, following surgical removal of both lesions, a histological diagnosis of SSP-MMNST was obtained.
Results
The literature review identified 21 eligible studies assessing 23 patients with SSP-MMNST, with a mean onset age of 41 years and a slight male gender preference. The lumbar district was the most involved spinal segment. Gross-total resection (GTR) was the treatment of choice in all amenable cases, followed in selected cases with residual tumor by adjuvant radiotherapy or chemotherapy. The metastatic and recurrence rates were 31.58% and 36.8%, respectively.
Conclusion
Differently from common schwannomas, MMNST represents a rare disease with known recurrence and metastatization propensity. As reported in our review, SSP-MMNST has a greater recurrence rate when compared to other forms of spinal MMNST, raising questions about the greater aggressiveness of the former. We also found that residual disease is related to a higher risk of systemic disease spreading. This metastatic potential, usually associated with primary lumbar localization, is characterized by a slight male prevalence. Indeed, whenever GTR is unachievable, considering the higher recurrence rate, adjuvant radiation therapy should be taken into consideration.
Melanotic malignant peripheral nerve sheath tumor is an extremely rare tumor, which originates from the neural crest, and more than half the cases are associated with Carney complex (myxomas, spotty pigmentation, and endocrine abnormalities). Herein, we have presented a case of a melanotic malignant peripheral nerve sheath tumor, which is not associated with Carney complex. The patient underwent preoperative nonenhanced CT, contrast-enhanced CT, and F-FDG PET/CT scans, which showed a large pelvic tumor with heterogeneous enhancement and increased F-FDG uptake. Subsequently, the patient underwent complete resection of the tumor.
Background. Psammomatous melanotic schwannoma is a rare neoplasm that may arise in the context of the Carney complex, a unique multiple endocrine neoplasia syndrome. Case. We herein report a case of psammomatous melanotic schwannoma not associated with the Carney complex, arising in the right paraspinal area of a 26-year-old woman. She underwent en bloc total resection of the mass and has exhibited a good clinical course for 10 years since tumor resection. Conclusion. Most melanotic schwannomas are slow-growing tumors; however, the prognosis can be unfavorable in cases with local recurrence or malignant behavior. This type of tumor is curable via resection and should be distinguished from other pigmented tumors, such as malignant melanoma.
