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Paraneoplastic cerebellar degeneration associated with noncutaneous Merkel cell carcinoma.

Zhang C, Emery L, Lancaster E - Neurol Neuroimmunol Neuroinflamm (2014)

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology (C.Z., E.L.) and Department of Pathology and Laboratory Medicine (L.E.), Hospital of the University of Pennsylvania, Philadelphia.

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Paraneoplastic cerebellar degeneration (PCD) is a rare condition that often heralds an underlying tumor... The syndrome is often associated with small cell carcinoma of the lung, breast cancer, gynecologic cancers, and Hodgkin disease... Antibodies to Hu, Yo, Ri, and CAR were negative... Electrophysiologic studies did not show evidence of Lambert-Eaton myasthenic syndrome (LEMS)... After tumor resection, he had 3 monthly IVIg treatments; however, his symptoms persisted... A 3-month repeat CT chest/abdomen/pelvis and colonoscopy did not show recurrent disease... The association between Merkel cell carcinoma and PCD was reported in 2005, although the specific antibody had not yet been identified... Two cases of Merkel cell carcinoma associated with LEMS have been described... LEMS is associated with antibodies to Cav2.1 VGCC... In PCD with VGCC antibodies, an underlying tumor, typically small cell carcinoma of the lung, is detected in approximately one-third of patients... This case highlights several important diagnostic and therapeutic considerations for patients with PCD... However, responses to therapy are often incomplete... Merkel cell tumors, although rare, may be particularly associated with VGCC antibodies, which may be associated with LEMS and/or PCD.

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Related in: MedlinePlus

Four months after symptom onset(A) Two fluorodeoxyglucose positive left inguinal lymph nodes. (B) CT pelvis showing 2 enlarged inguinal lymph nodes: posterior node is 24 × 19 mm, anterior node is 16 × 13 mm. (C) Inguinal lymphadenectomy showing high-grade neuroendocrine carcinoma (hematoxylin & eosin staining, 400× magnification). (D) Immunohistochemical staining for CK20 showing perinuclear “dot-like” staining, characteristic of Merkel cell carcinoma (400× magnification).
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Figure 1: Four months after symptom onset(A) Two fluorodeoxyglucose positive left inguinal lymph nodes. (B) CT pelvis showing 2 enlarged inguinal lymph nodes: posterior node is 24 × 19 mm, anterior node is 16 × 13 mm. (C) Inguinal lymphadenectomy showing high-grade neuroendocrine carcinoma (hematoxylin & eosin staining, 400× magnification). (D) Immunohistochemical staining for CK20 showing perinuclear “dot-like” staining, characteristic of Merkel cell carcinoma (400× magnification).

Mentions: At 2-month follow-up, his ataxia was severe. Repeat MRI brain was unremarkable, and repeat lumbar puncture showed resolution of the leukocytosis. However, a paraneoplastic panel (Athena Diagnostics, Worcester, MA) from his CSF showed elevated levels of P/Q-type VGCC antibodies to 220 pmol/L. Antibodies to Hu, Yo, Ri, and CAR were negative. Electrophysiologic studies did not show evidence of Lambert-Eaton myasthenic syndrome (LEMS). He was started on high-dose oral steroids. A PET scan revealed hypermetabolic lesions in the descending colon and left inguinal lymph nodes (figure, A and B). Meanwhile, his function and neurologic examination worsened so he was started on IV immunoglobulin (IVIg) (0.4 g/kg for 5 days). Colonoscopy demonstrated an adenomatous polyp and high-grade dysplasia in the descending colon. Excisional biopsy of his left inguinal node revealed high-grade neuroendocrine carcinoma with tumor cells expressing Pan-CK, CK20 (perinuclear dot-like), chromogranin, CD56, and Ki-67, consistent with Merkel cell carcinoma (figure, C and D). Subsequent lymphadenectomy demonstrated metastatic carcinoma involving 1 of 14 nodes without extracapsular extension. He was diagnosed with Merkel cell carcinoma stage IIIA (T0N1aM0) with unknown primary. His dermatologic examination was unremarkable.


Paraneoplastic cerebellar degeneration associated with noncutaneous Merkel cell carcinoma.

Zhang C, Emery L, Lancaster E - Neurol Neuroimmunol Neuroinflamm (2014)

Four months after symptom onset(A) Two fluorodeoxyglucose positive left inguinal lymph nodes. (B) CT pelvis showing 2 enlarged inguinal lymph nodes: posterior node is 24 × 19 mm, anterior node is 16 × 13 mm. (C) Inguinal lymphadenectomy showing high-grade neuroendocrine carcinoma (hematoxylin & eosin staining, 400× magnification). (D) Immunohistochemical staining for CK20 showing perinuclear “dot-like” staining, characteristic of Merkel cell carcinoma (400× magnification).
© Copyright Policy - open-access
Related In: Results  -  Collection

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Figure 1: Four months after symptom onset(A) Two fluorodeoxyglucose positive left inguinal lymph nodes. (B) CT pelvis showing 2 enlarged inguinal lymph nodes: posterior node is 24 × 19 mm, anterior node is 16 × 13 mm. (C) Inguinal lymphadenectomy showing high-grade neuroendocrine carcinoma (hematoxylin & eosin staining, 400× magnification). (D) Immunohistochemical staining for CK20 showing perinuclear “dot-like” staining, characteristic of Merkel cell carcinoma (400× magnification).
Mentions: At 2-month follow-up, his ataxia was severe. Repeat MRI brain was unremarkable, and repeat lumbar puncture showed resolution of the leukocytosis. However, a paraneoplastic panel (Athena Diagnostics, Worcester, MA) from his CSF showed elevated levels of P/Q-type VGCC antibodies to 220 pmol/L. Antibodies to Hu, Yo, Ri, and CAR were negative. Electrophysiologic studies did not show evidence of Lambert-Eaton myasthenic syndrome (LEMS). He was started on high-dose oral steroids. A PET scan revealed hypermetabolic lesions in the descending colon and left inguinal lymph nodes (figure, A and B). Meanwhile, his function and neurologic examination worsened so he was started on IV immunoglobulin (IVIg) (0.4 g/kg for 5 days). Colonoscopy demonstrated an adenomatous polyp and high-grade dysplasia in the descending colon. Excisional biopsy of his left inguinal node revealed high-grade neuroendocrine carcinoma with tumor cells expressing Pan-CK, CK20 (perinuclear dot-like), chromogranin, CD56, and Ki-67, consistent with Merkel cell carcinoma (figure, C and D). Subsequent lymphadenectomy demonstrated metastatic carcinoma involving 1 of 14 nodes without extracapsular extension. He was diagnosed with Merkel cell carcinoma stage IIIA (T0N1aM0) with unknown primary. His dermatologic examination was unremarkable.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology (C.Z., E.L.) and Department of Pathology and Laboratory Medicine (L.E.), Hospital of the University of Pennsylvania, Philadelphia.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Paraneoplastic cerebellar degeneration (PCD) is a rare condition that often heralds an underlying tumor... The syndrome is often associated with small cell carcinoma of the lung, breast cancer, gynecologic cancers, and Hodgkin disease... Antibodies to Hu, Yo, Ri, and CAR were negative... Electrophysiologic studies did not show evidence of Lambert-Eaton myasthenic syndrome (LEMS)... After tumor resection, he had 3 monthly IVIg treatments; however, his symptoms persisted... A 3-month repeat CT chest/abdomen/pelvis and colonoscopy did not show recurrent disease... The association between Merkel cell carcinoma and PCD was reported in 2005, although the specific antibody had not yet been identified... Two cases of Merkel cell carcinoma associated with LEMS have been described... LEMS is associated with antibodies to Cav2.1 VGCC... In PCD with VGCC antibodies, an underlying tumor, typically small cell carcinoma of the lung, is detected in approximately one-third of patients... This case highlights several important diagnostic and therapeutic considerations for patients with PCD... However, responses to therapy are often incomplete... Merkel cell tumors, although rare, may be particularly associated with VGCC antibodies, which may be associated with LEMS and/or PCD.

No MeSH data available.


Related in: MedlinePlus