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Metastatic extrapulmonary small cell carcinoma to the cerebellopontine angle: a case report and review of the literature.

Theodros D, Goodwin CR, Crane GM, Liauw J, Kleinberg L, Lim M - Case Rep Oncol Med (2015)

Bottom Line: Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm.Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease.The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, The Johns Hopkins Hospital, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.

ABSTRACT
Extrapulmonary small cell carcinomas (EPSCC) are rare malignancies with poor patient prognoses. We present the case of a 63-year-old male who underwent surgical resection of a poorly differentiated small cell carcinoma, likely from a small intestinal primary tumor that metastasized to the cerebellopontine angle (CPA). A 63-year-old male presented with mild left facial paralysis, hearing loss, and balance instability. MRI revealed a 15 mm mass in the left CPA involving the internal auditory canal consistent with a vestibular schwannoma. Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm. The patient underwent a suboccipital craniectomy and the mass was grossly different visually and in consistency from a standard vestibular schwannoma. The final pathology revealed a poorly differentiated small cell carcinoma. Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease. The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms. Surgical resection and radiotherapy are potential treatment options to improve survival in patients diagnosed with NET brain metastases. We present the first documented case of skull base metastasis of a poorly differentiated small cell carcinoma involving the CPA.

No MeSH data available.


Related in: MedlinePlus

(a) T1-axial MRI with contrast and (b) T2-axial Flair MRI performed at initial encounter demonstrate a minimally enhancing mass in the left cerebellopontine angle.
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fig1: (a) T1-axial MRI with contrast and (b) T2-axial Flair MRI performed at initial encounter demonstrate a minimally enhancing mass in the left cerebellopontine angle.

Mentions: A 63-year-old man with a past medical history of hypertension, hyperlipidemia, hypothyroidism, and gout presented with left facial paralysis, dryness and irritation in his left eye, hearing loss, and balance instability that occurred over the course of weeks. The patient was initially diagnosed with benign paroxysmal positional vertigo and started on meclizine with no relief. He was referred to an otolaryngologist and magnetic resonance imaging (MRI) was performed at an outside institution that demonstrated a 15 mm enhancing mass in the left CPA with involvement of the internal auditory canal that was most consistent with a vestibular schwannoma. The patient was counseled as to the different treatment options for a presumed vestibular schwannoma with repeat imaging performed at our institution and chose surgery (Figure 1). Eight weeks later, the patient began experiencing worsening facial function, was classified as a House-Brackmann VI on physical examination, and also reported difficulty with swallowing. A repeat MRI revealed the mass had increased to 35 mm with mass effect on the pons, superior, middle, and inferior cerebellar peduncles with associated edema (Figure 2). The patient subsequently underwent a left-sided retrosigmoid craniotomy. Intraoperatively, the dura and the tumor tissue itself were erythematous, friable, and extremely hemorrhagic. The mass also had an unexpected consistency and appearance as it was very soft, friable, and purplish in color. Preliminary frozen specimens sent to pathology were concerning for lymphoma. The patient's tumor was then debulked. Post-operatively, the patient underwent a whole body PET scan, which demonstrated high FDG activity in the location of the known residual left CPA mass, as well as an additional hypermetabolic mesenteric lesion abutting the ileum and ascending colon in the right lower quadrant (Figure 2). The final pathology report identified the CPA lesion as a poorly differentiated small cell neuroendocrine carcinoma and was confirmed by positive immunostaining of the tumor cells for cytokeratin CAM 5.2, synaptophysin, and CD56 and a high proliferative index (Figure 3). The tumor cells were negative for CD3, CD20, Epstein-Barr virus, S100, and thyroid transcription factor-1 (TTF-1). The mesenteric lesion was not biopsied at this time. The patient continued to experience symptoms of left facial paralysis, left eye irritation, decreased hearing loss, imbalance, and vertigo postoperatively but was discharged home in good condition. He underwent whole brain radiation therapy followed by chemotherapy and at last follow-up postoperatively has continued to show signs of improvement (Figure 4).


Metastatic extrapulmonary small cell carcinoma to the cerebellopontine angle: a case report and review of the literature.

Theodros D, Goodwin CR, Crane GM, Liauw J, Kleinberg L, Lim M - Case Rep Oncol Med (2015)

(a) T1-axial MRI with contrast and (b) T2-axial Flair MRI performed at initial encounter demonstrate a minimally enhancing mass in the left cerebellopontine angle.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4355812&req=5

fig1: (a) T1-axial MRI with contrast and (b) T2-axial Flair MRI performed at initial encounter demonstrate a minimally enhancing mass in the left cerebellopontine angle.
Mentions: A 63-year-old man with a past medical history of hypertension, hyperlipidemia, hypothyroidism, and gout presented with left facial paralysis, dryness and irritation in his left eye, hearing loss, and balance instability that occurred over the course of weeks. The patient was initially diagnosed with benign paroxysmal positional vertigo and started on meclizine with no relief. He was referred to an otolaryngologist and magnetic resonance imaging (MRI) was performed at an outside institution that demonstrated a 15 mm enhancing mass in the left CPA with involvement of the internal auditory canal that was most consistent with a vestibular schwannoma. The patient was counseled as to the different treatment options for a presumed vestibular schwannoma with repeat imaging performed at our institution and chose surgery (Figure 1). Eight weeks later, the patient began experiencing worsening facial function, was classified as a House-Brackmann VI on physical examination, and also reported difficulty with swallowing. A repeat MRI revealed the mass had increased to 35 mm with mass effect on the pons, superior, middle, and inferior cerebellar peduncles with associated edema (Figure 2). The patient subsequently underwent a left-sided retrosigmoid craniotomy. Intraoperatively, the dura and the tumor tissue itself were erythematous, friable, and extremely hemorrhagic. The mass also had an unexpected consistency and appearance as it was very soft, friable, and purplish in color. Preliminary frozen specimens sent to pathology were concerning for lymphoma. The patient's tumor was then debulked. Post-operatively, the patient underwent a whole body PET scan, which demonstrated high FDG activity in the location of the known residual left CPA mass, as well as an additional hypermetabolic mesenteric lesion abutting the ileum and ascending colon in the right lower quadrant (Figure 2). The final pathology report identified the CPA lesion as a poorly differentiated small cell neuroendocrine carcinoma and was confirmed by positive immunostaining of the tumor cells for cytokeratin CAM 5.2, synaptophysin, and CD56 and a high proliferative index (Figure 3). The tumor cells were negative for CD3, CD20, Epstein-Barr virus, S100, and thyroid transcription factor-1 (TTF-1). The mesenteric lesion was not biopsied at this time. The patient continued to experience symptoms of left facial paralysis, left eye irritation, decreased hearing loss, imbalance, and vertigo postoperatively but was discharged home in good condition. He underwent whole brain radiation therapy followed by chemotherapy and at last follow-up postoperatively has continued to show signs of improvement (Figure 4).

Bottom Line: Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm.Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease.The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, The Johns Hopkins Hospital, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.

ABSTRACT
Extrapulmonary small cell carcinomas (EPSCC) are rare malignancies with poor patient prognoses. We present the case of a 63-year-old male who underwent surgical resection of a poorly differentiated small cell carcinoma, likely from a small intestinal primary tumor that metastasized to the cerebellopontine angle (CPA). A 63-year-old male presented with mild left facial paralysis, hearing loss, and balance instability. MRI revealed a 15 mm mass in the left CPA involving the internal auditory canal consistent with a vestibular schwannoma. Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm. The patient underwent a suboccipital craniectomy and the mass was grossly different visually and in consistency from a standard vestibular schwannoma. The final pathology revealed a poorly differentiated small cell carcinoma. Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease. The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms. Surgical resection and radiotherapy are potential treatment options to improve survival in patients diagnosed with NET brain metastases. We present the first documented case of skull base metastasis of a poorly differentiated small cell carcinoma involving the CPA.

No MeSH data available.


Related in: MedlinePlus