Recurrence of esophageal carcinoma presenting as dermatomyositis

A 55-year-old male was diagnosed of squamous cell carcinoma of esophagus and was treated by surgical resection of the tumor. Fluorodeoxyglucose (FDG) positron emission tomography performed before surgery did not show any evidence of local or distant lymph node metastasis. The patient was not given adjunctive chemotherapy or radiotherapy. Seven months later he presented with dermatomyositis and on evaluation was found to have metastatic deposit of squamous cell carcinoma in cervical lymph nodes. Despite steroids and radiotherapy he died within 2 months of diagnosis. We report the first case of dermatomyositis as a manifestation of recurrence of esophageal carcinoma.

Keywords: Creatinine phosphokinase, dermatomyositis, 18 florodeoxyglucose positron emission tomography, squamous cell carcinoma

The relation between dermatomyositis and malignancy is well known. ^[1] It usually precedes the diagnosis of malignancy; however, rarely it can manifest after the diagnosis of malignancy. It has also been associated with recurrence of malignancies especially breast, ovarian, and lung carcinomas. We, hereby, report the first case of dermatomyositis presenting as recurrence of esophageal carcinoma.

A 55-year-old male presented to the emergency department with 4-weeks history of generalized rash and swelling of hand and feet. He also complained of difficulty in climbing stairs, getting up from sitting position and performing his day-to-day routine work. The patient was operated 7 months back for esophageal carcinoma (squamous cell carcinoma). Local or distant metastasis to lymph nodes or other structures was ruled out by preoperative fluorine 18 fluorodeoxyglucose (FDG) positron emission tomography (PET) which confirmed only local disease. The patient was neither given radiotherapy nor chemotherapy. On examination the patient had rash involving face, upper eyelid, periorbital region, forehead, forearm, hand, feet, and legs. There was also puffiness of hands and feet. The rash was intensely pruritic. The pulse was 84 beats/min, BP 130/80 mmHg, a small lymph node (1.5 × 1.0 cm) hard, non-mobile was palpable in the left supraclavicular area. Examination of the chest, CVS, and abdomen was normal. CNS examination revealed grade 4 minus power all over with proximal muscle weakness.

Investigations revealed Hb 9.2 g/dL, total leucocyte count (TLC) 5.4 × 10 ⁹ / L; Differential leucocyte count (DLC) N 67%, L 31%, M 2%; Plt 149 × 10 ⁹ /L; erythrocyte sedimentation rate (ESR) 30 mm /hr; urea 40 mg/dL; creatinine 1.2 mg/dL; sugar (random) 112 mg/dL; bilirubin 1.35 mg/dL; aspartate aminotransferase (AST) 74 U/L; alanine aminotransferase (ALT) 68 U/L; alkaline phosphatase (ALP) 210 U/L; total protein 6.8 g/dL; albumin 3.6 g/dL; CPK 784 U/L, calcium 9.4 mg/dL; phosphorus 3.2 mg/dL; uric acid 6.0 mg/dL; lactate dehydrogenase (LDH) 464 U/L. ECG, chest X-ray, and USG abdomen were normal. Electromyography (EMG) was myopathic pattern showing positive sharp waves, fibrillations, and increased insertional activity with spontaneous high-frequency discharges. The patient refused muscle biopsy. Endoscopic gastroduodenoscopy (EGD) showed mild erythema at anastamosis site; however, no biopsy was taken. CT chest showed lymph nodes in the mediastinum. CT abdomen was normal. FNAC of the supraclavicular lymph node showed metastatic deposits of squamous cell carcinoma. The patient was diagnosed as having dermatomyositis on the basis of symmetrical weakness of the limb girdle muscles, elevated CPK, AST, ALT, LDH, and myopathic EMG associated with rash consistent with dermatomyositis. The patient was started on high-dose steroids and radiotherapy. He showed some initial improvement; however, later he succumbed to respiratory tract infection.

Dermatomyositis is characterized by a specific rash, symmetrical proximal muscle weakness, elevated creatinine phosphokinase, abnormal electromyography, and abnormal muscle biopsy. ^[2]

Dermatomyositis is known to be associated with malignancy and the reported incidence varies from 7% to 34%. ^[1] It usually precedes lung, breast, and ovarian malignancies. ^{[3],[4],[5],[6]} It has been usually associated with lung cancer in men and ovarian cancer in women. ^[7],[8] Although less common dermatomyositis has been associated with esophageal carcinomas. ^{[9],[10],[11],[12],[13]} The occurrence of dermatomyositis usually precedes malignancy; however, it can occur any time. It has been associated with the recurrence of lung, breast, and ovarian carcinomas. ^{[14],[15],[16]}

The most important prognostic factor in esophageal carcinoma is metastasis to the lymph nodes. ^[17] The number and location of involved lymph nodes are also considered as important prognostic factors. ^[18] The major cause of death in esophageal carcinoma is the local recurrence which can go up to 80%. ^[19] Up to 50% of these recurrences are due to metastasis in lymph nodes left after surgery. ^[19] Although FDG-PET is being utilized increasingly for preoperative staging of esophageal carcinomas it also has limitations as it cannot detect micrometastasis to lymph nodes due to its limited resolution and scatter effects. ^{[18],[20],[21]} The metastatic deposits of squamous cell carcinoma in the supraclavicular lymph nodes are related to the recurrence of esophageal carcinoma in our case as the disease commonly spreads to adjacent and supraclavicular lymph nodes. Also dermatomyositis as a manifestation of metastatic esophageal adenocarcinoma has been reported earlier. ^[10]

Hence it is imperative for a clinician to be aware of the limitations of FDG-PET and association of dermatomyositis with esophageal carcinoma recurrence.

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1755-6783.127807