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Persistent primary cutaneous primitive neuroectodermal tumor 4 years after chemotherapy
J AM ACAD DERMATOL
440 Letters
AUGUST 2011
REFERENCES 1. Cheshire WP, Freeman R. Disorders of sweating. Semin Neurol 2003;23:399-406. 2. Mu~ noz-P erez MA, Mazuecos J, Ortega M, Camacho F. Guess what! Unilateral anhidrosis: first clinical manifestation of bronchial carcinoma. Eur J Dermatol 2001;11:257-8. 3. Ficker JH. Unilateral anhidrosis of the leg. Lancet 2002;360:129. 4. Slabbynck H, Bedert L, De Deyn PP, Galdermans D, Coolen D. Unilateral segmental hyperhidrosis associated with pulmonary adenocarcinoma. Chest 1998;114:1215-7. 5. Tascilar N, Tekin NS, Erdem Z, Alpay A, Emre U. Unnoticed dysautonomic syndrome of the face: Harlequin syndrome. Auton Neurosci 2007;137:1-9. doi:10.1016/j.jaad.2010.01.043
Fig 1. Fleshy cystic nodule representing cutaneous primitive neuroectodermal tumor.
Persistent primary cutaneous primitive neuroectodermal tumor 4 years after chemotherapy To the Editor: A 52-year-old black woman presented with a 3-month history of a painless, 3 cm, soft nodule on the scalp (Fig 1). She reported that this cyst was in the same location as an unknown type of tumor that she had been treated for more than 4 years prior. She underwent biopsy of this cyst which showed a grossly hemorrhagic, fragmented mass, which was shelled out. On hematoxylin-eosin staining, spoke-wheel arrangements of small round, blue cells with wedge-shaped cytoplasm surrounding a central core of cytoplasmic processes, were visible (Fig 2). Immunohistochemical analysis was positive for neuron-specific enolase and focally positive for S-100 protein. Reverse transcriptase polymerase chain reaction analysis demonstrated expression of EWS/FLI-1 fusion gene mRNA transcripts. This combination of histopathologic, immunohistochemical, and genetic findings is definitive for primitive neuroectodermal tumor (PNET). Upon obtaining her outside medical records it was found she had completed treatment for a primary cutaneous PNET at this site on the scalp 4.5 years earlier with multiple rounds of systemic ifosfamide, vincristine, doxorubicin, and cyclophosphamide. We referred her to oncology for additional evaluation and management. She had no evidence of systemic disease and was diagnosed with local recurrence of primary cutaneous PNET. Treatment consisted of 8 cycles of chemotherapy with ifosfamide, vincristine, doxorubicin, and cyclophosphamide, followed by local radiation treatments. The patient remains without evidence of subsequent recurrence 40 months after completing this treatment for recurrent disease. Primary cutaneous PNET is a rare disease with fewer than 50 identified cases.1 Most PNET cases
Fig 2. Proliferation of small round blue cells with Homer Wright rosette formations. (Hematoxylin-eosin stain; original magnification: 340.)
occur in Caucasians and are very rare in blacks. Treatment may include combinations of surgery, chemotherapy, and sometimes radiation.2 Prognosis of primary cutaneous PNET is good, with the exception of a few cases.1,3-5 This is in contrast to other forms of PNET, such as those occurring in bone or deep soft tissue, which have much lower survival outcomes. This has led some to speculate that primary cutaneous PNET has a different clinical behavior and can be treated distinctly from other systemic forms of PNET. The prognosis for primary cutaneous PNET has thus far not been linked to variation in treatment modalities. To our knowledge, no other cases of local cutaneous reappearance after chemotherapy have been reported. We report this case to identify a variation in clinical behavior for this rare tumor. Long-term clinical follow-up in cutaneous PNET cases is limited. Additional analyses may help to improve understanding of the clinical behavior of PNET as well as to standardize treatment recommendations for primary cutaneous PNET.
J AM ACAD DERMATOL
Letters 441
VOLUME 65, NUMBER 2
L. Katie Morrison, MD,a H. Nicholas Shamma, MD,b and Michael P. Heffernan, MDc University of Texas Medical Center, Department of Dermatology, Houston, Texas,a and Dermatopathology Laboratory of Central Statesb; and Central Dermatology, St Louis, Missouric Funding sources: None. Conflicts of interest: None declared. Reprint requests: L. Katie Morrison, MD, 6655 Travis, Suite 980, Houston, TX 77030 E-mail: [email protected] REFERENCES 1. Terrier-Lacombe MJ, et al. Superficial primitive Ewing’s sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases. Mod Pathol 2009;22:87-94. 2. Paulussen M, et al. Ewing’s sarcoma of the bone: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 2008;19(Suppl 2):ii97-8. 3. Ehrig T, Billings SD, Fanburg-Smith JC. Superficial primitive neuroectodermal tumor/Ewing sarcoma (PN/ES): same tumor as deep PN/ES or new entity? Ann Diagn Pathol 2007;11:153-9. 4. Banerjee SS, et al. Clinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue. Histopathology 1997;31:355-66. 5. Chow E, et al. Cutaneous and subcutaneous Ewing’s sarcoma: an indolent disease. Int J Radiat Oncol Biol Phys 2000;46:433-8. doi:10.1016/j.jaad.2010.01.059
Bipolar forceps: A hemostatic tool for patients with electrocoagulation-induced dental pain To the Editor: We describe a case of electrocoagulation-induced tooth pain despite appropriate surgical technique and local anesthesia, which resolved upon switching to the use of bipolar forceps for hemostasis. A 52-year-old woman underwent Mohs micrographic surgery of a biopsy-proven nodular basal cell carcinoma on the left nasal sidewall. The skin surrounding the lesion was anesthetized with 3 mL of 1% lidocaine with 1:100,000 of epinephrine. The tumor was excised in one stage without difficulty; however, during the repair process the patient experienced a ‘‘shock-like’’ sensation localized to her teeth and gums immediately upon biterminal monopolar electrocoagulation for hemostasis. The sensation was in the area of her metal dental fillings in the left upper dentition. Despite injection of an additional 1 mL of lidocaine with epinephrine, the patient reported persistence of the electrical shockelike sensation upon electrocoagulation. The monopolar device was replaced with an
electrosurgical device with bipolar forceps, and hemostasis was achieved without symptom recurrence. Electrosurgical units such as the one used in this case require electrical current to establish hemostasis or other desired tissue effects, such as cutting. The tissue is heated by a current which originates from an unheated probe tip and passes through the body to a dispersive ( grounding) pad or electrode before returning to the electrosurgical unit.1,2 Electrocoagulation-induced tooth pain during Mohs micrographic surgery related to metal dental restoration has been reported previously in a single case report,3 and we have on rare occasions witnessed this phenomenon in our own practice. It is likely that metal dental restoration acts as a conductor in the circuit, and the shock sensation is sensed in the surrounding tissue where the current enters and exits. Bipolar devices differ from monopolar devices in that they concentrate the current across the electrode prongs rather than dispersing the energy through the body. In the bipolar mode, current is directed from the active prong to the dispersive prong.1 Bipolar devices are preferred to monopolar devices in patients with pacemakers and defibrillators because the bipolar device limits the current to a focal area and thereby minimizes interference.4 Likewise, because bipolar devices minimize the path that the current takes through the body, they have less tendency to cause fasciculations of nearby muscles or to stimulate sensory nerves as compared with monopolar devices.1 In the above-described patient case, changing to bipolar forceps allowed us to achieve hemostasis without discomfort. On the basis of our experience and knowledge of the various modes of electrosurgery, we believe that bipolar forceps may be used effectively to comfortably provide hemostasis in patients who experience electrical sensations during procedures with monopolar biterminal electrocoagulation. Monoterminal electrodessication has also been reported as a safe alternative.3 Yolanda Lenzy, MD,a Deborah L. Cummins, MD,b and Daniel T. Finn, MDc Dermatology, Boston University/Tufts Medical Center Combined Programa; Dermatologic Surgery, Tufts Medical Centerb; Dermatology and Dermatologic Surgery, Director of Satellite Surgical Program, Tufts Medical Center,c Boston, Massachusetts Funding sources: None. Conflicts of interest: None declared.
440 Letters
AUGUST 2011
REFERENCES 1. Cheshire WP, Freeman R. Disorders of sweating. Semin Neurol 2003;23:399-406. 2. Mu~ noz-P erez MA, Mazuecos J, Ortega M, Camacho F. Guess what! Unilateral anhidrosis: first clinical manifestation of bronchial carcinoma. Eur J Dermatol 2001;11:257-8. 3. Ficker JH. Unilateral anhidrosis of the leg. Lancet 2002;360:129. 4. Slabbynck H, Bedert L, De Deyn PP, Galdermans D, Coolen D. Unilateral segmental hyperhidrosis associated with pulmonary adenocarcinoma. Chest 1998;114:1215-7. 5. Tascilar N, Tekin NS, Erdem Z, Alpay A, Emre U. Unnoticed dysautonomic syndrome of the face: Harlequin syndrome. Auton Neurosci 2007;137:1-9. doi:10.1016/j.jaad.2010.01.043
Fig 1. Fleshy cystic nodule representing cutaneous primitive neuroectodermal tumor.
Persistent primary cutaneous primitive neuroectodermal tumor 4 years after chemotherapy To the Editor: A 52-year-old black woman presented with a 3-month history of a painless, 3 cm, soft nodule on the scalp (Fig 1). She reported that this cyst was in the same location as an unknown type of tumor that she had been treated for more than 4 years prior. She underwent biopsy of this cyst which showed a grossly hemorrhagic, fragmented mass, which was shelled out. On hematoxylin-eosin staining, spoke-wheel arrangements of small round, blue cells with wedge-shaped cytoplasm surrounding a central core of cytoplasmic processes, were visible (Fig 2). Immunohistochemical analysis was positive for neuron-specific enolase and focally positive for S-100 protein. Reverse transcriptase polymerase chain reaction analysis demonstrated expression of EWS/FLI-1 fusion gene mRNA transcripts. This combination of histopathologic, immunohistochemical, and genetic findings is definitive for primitive neuroectodermal tumor (PNET). Upon obtaining her outside medical records it was found she had completed treatment for a primary cutaneous PNET at this site on the scalp 4.5 years earlier with multiple rounds of systemic ifosfamide, vincristine, doxorubicin, and cyclophosphamide. We referred her to oncology for additional evaluation and management. She had no evidence of systemic disease and was diagnosed with local recurrence of primary cutaneous PNET. Treatment consisted of 8 cycles of chemotherapy with ifosfamide, vincristine, doxorubicin, and cyclophosphamide, followed by local radiation treatments. The patient remains without evidence of subsequent recurrence 40 months after completing this treatment for recurrent disease. Primary cutaneous PNET is a rare disease with fewer than 50 identified cases.1 Most PNET cases
Fig 2. Proliferation of small round blue cells with Homer Wright rosette formations. (Hematoxylin-eosin stain; original magnification: 340.)
occur in Caucasians and are very rare in blacks. Treatment may include combinations of surgery, chemotherapy, and sometimes radiation.2 Prognosis of primary cutaneous PNET is good, with the exception of a few cases.1,3-5 This is in contrast to other forms of PNET, such as those occurring in bone or deep soft tissue, which have much lower survival outcomes. This has led some to speculate that primary cutaneous PNET has a different clinical behavior and can be treated distinctly from other systemic forms of PNET. The prognosis for primary cutaneous PNET has thus far not been linked to variation in treatment modalities. To our knowledge, no other cases of local cutaneous reappearance after chemotherapy have been reported. We report this case to identify a variation in clinical behavior for this rare tumor. Long-term clinical follow-up in cutaneous PNET cases is limited. Additional analyses may help to improve understanding of the clinical behavior of PNET as well as to standardize treatment recommendations for primary cutaneous PNET.
J AM ACAD DERMATOL
Letters 441
VOLUME 65, NUMBER 2
L. Katie Morrison, MD,a H. Nicholas Shamma, MD,b and Michael P. Heffernan, MDc University of Texas Medical Center, Department of Dermatology, Houston, Texas,a and Dermatopathology Laboratory of Central Statesb; and Central Dermatology, St Louis, Missouric Funding sources: None. Conflicts of interest: None declared. Reprint requests: L. Katie Morrison, MD, 6655 Travis, Suite 980, Houston, TX 77030 E-mail: [email protected] REFERENCES 1. Terrier-Lacombe MJ, et al. Superficial primitive Ewing’s sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases. Mod Pathol 2009;22:87-94. 2. Paulussen M, et al. Ewing’s sarcoma of the bone: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 2008;19(Suppl 2):ii97-8. 3. Ehrig T, Billings SD, Fanburg-Smith JC. Superficial primitive neuroectodermal tumor/Ewing sarcoma (PN/ES): same tumor as deep PN/ES or new entity? Ann Diagn Pathol 2007;11:153-9. 4. Banerjee SS, et al. Clinicopathological characteristics of peripheral primitive neuroectodermal tumour of skin and subcutaneous tissue. Histopathology 1997;31:355-66. 5. Chow E, et al. Cutaneous and subcutaneous Ewing’s sarcoma: an indolent disease. Int J Radiat Oncol Biol Phys 2000;46:433-8. doi:10.1016/j.jaad.2010.01.059
Bipolar forceps: A hemostatic tool for patients with electrocoagulation-induced dental pain To the Editor: We describe a case of electrocoagulation-induced tooth pain despite appropriate surgical technique and local anesthesia, which resolved upon switching to the use of bipolar forceps for hemostasis. A 52-year-old woman underwent Mohs micrographic surgery of a biopsy-proven nodular basal cell carcinoma on the left nasal sidewall. The skin surrounding the lesion was anesthetized with 3 mL of 1% lidocaine with 1:100,000 of epinephrine. The tumor was excised in one stage without difficulty; however, during the repair process the patient experienced a ‘‘shock-like’’ sensation localized to her teeth and gums immediately upon biterminal monopolar electrocoagulation for hemostasis. The sensation was in the area of her metal dental fillings in the left upper dentition. Despite injection of an additional 1 mL of lidocaine with epinephrine, the patient reported persistence of the electrical shockelike sensation upon electrocoagulation. The monopolar device was replaced with an
electrosurgical device with bipolar forceps, and hemostasis was achieved without symptom recurrence. Electrosurgical units such as the one used in this case require electrical current to establish hemostasis or other desired tissue effects, such as cutting. The tissue is heated by a current which originates from an unheated probe tip and passes through the body to a dispersive ( grounding) pad or electrode before returning to the electrosurgical unit.1,2 Electrocoagulation-induced tooth pain during Mohs micrographic surgery related to metal dental restoration has been reported previously in a single case report,3 and we have on rare occasions witnessed this phenomenon in our own practice. It is likely that metal dental restoration acts as a conductor in the circuit, and the shock sensation is sensed in the surrounding tissue where the current enters and exits. Bipolar devices differ from monopolar devices in that they concentrate the current across the electrode prongs rather than dispersing the energy through the body. In the bipolar mode, current is directed from the active prong to the dispersive prong.1 Bipolar devices are preferred to monopolar devices in patients with pacemakers and defibrillators because the bipolar device limits the current to a focal area and thereby minimizes interference.4 Likewise, because bipolar devices minimize the path that the current takes through the body, they have less tendency to cause fasciculations of nearby muscles or to stimulate sensory nerves as compared with monopolar devices.1 In the above-described patient case, changing to bipolar forceps allowed us to achieve hemostasis without discomfort. On the basis of our experience and knowledge of the various modes of electrosurgery, we believe that bipolar forceps may be used effectively to comfortably provide hemostasis in patients who experience electrical sensations during procedures with monopolar biterminal electrocoagulation. Monoterminal electrodessication has also been reported as a safe alternative.3 Yolanda Lenzy, MD,a Deborah L. Cummins, MD,b and Daniel T. Finn, MDc Dermatology, Boston University/Tufts Medical Center Combined Programa; Dermatologic Surgery, Tufts Medical Centerb; Dermatology and Dermatologic Surgery, Director of Satellite Surgical Program, Tufts Medical Center,c Boston, Massachusetts Funding sources: None. Conflicts of interest: None declared.