Retroperitoneal Intramuscular Neuri- lemmoma - An Unusual - PDF document

Retroperitoneal Intramuscular Neuri- lemmoma - An Unusual Presentation TEJ KUMAR Y., SANTOSH KUMAR BEHERA, SUPREET KUMAR, NEMBIAN RAJA RAJAN B, SUKANTI MAJHI, ALPHONSE JITHIN ABSTRACT Neurilemmomas or Schwannomas originate from Schwann cells of the peripheral nerve fibers and are usually located in the head, neck, and flexor surfaces of the extremities. Primary tumors of the retroperitoneal region are quite rare and schwannomas comprise only 1–6% of them. They are insidious in onset and have nonspecific and misleading symptoms. It is difficult to diagnose them and it becomes more challenging if they undergo secondary changes. Here we present a case of 32 year old female with recurrent right abdominal pain, intermittent fever and pain in Right lower limb during walking for 6 months. Ultrasound revealed a Psoas Abscess that recurred after an initial attempt of aspiration of 30ml of clear fluid, CT diagnosis of Hydatid Cyst was made and on exploratory laparotomy a cystic swelling was found to arise from intramuscular plane of Psoas muscle which was excised along with its capsule which after Histopathological examination was found to be a Neurilemmoma. Literature review provides information about the rarity of the condition and information regarding the diagnosis and treatment of this unusual case. Key Words: Neurilemmoma, Schwannoma, Psoas Muscle, Hydatid Cyst Introduction be located in the retroperitoneal without any chills and rigors and region 3 And among schwannomas had an antalgic gait at the time of Neurilemmomas are tumors in retroperitoneum, 98% are benign presentation without there being arising from Schwann cells of and about 2 % are malignant and the any previous history of trauma. the peripheral nerve sheaths. They incidence of malignancy is more in No history of TB, DM and no are generally slow growing and schwannomas of retroperitoneum history of similar illness in any painless tumors and predominantly than the peripheral ones 4 Typically, family members. occur in females between the it is very difficult to diagnose 2nd and 5th decade of life. 1 On Examination of Abdomen retroperitoneal tumors before the Most schwannomas occur in the On Inspection: Abdomen was operation, since both clinical and cephalocervical region and limbs, scaphoid in shape, flanks were tumors in the retroperitoneal radiologic features specific to normal, a solitary lump visible in location are rare and comprise schwannomas are usually absent. Right Lumbar lesion approximately 6% of primary retroperitoneal Here we describe a rare case measuring 5x4 cm. neoplasms. 2 The majority of of retroperitoneal Intramuscular retroperitoneal schwannomas (Psoas Muscle) neurilemmoma. On Palpation: A swelling of are benign in nature although size 4x4 cm in Right lumbar Case Report malignant ones have also been region without any local rise A 32 year old female presented reported. Among all schwannomas, of temperature or tenderness. with chief complaints of Recurrent only 0.7% benign and 1.7% With smooth surface, ill defined RIGHT abdominal pain with malignant ones are reported to margins, firm consistency. It intermittent fever and pain in right was non pulsatile, non reducible, lower limb during walking for Tej Kumar Y., MS, FAIS, Associate Professor, non compressible without any Santosh Kumar Behera, MS, FIAGES, past 6 months. Pain started in the Assistant Professor, impulse on coughing, didn’t move right lumbar region, was insidious Supreet Kumar, with respiration, had restricted Junior Resident & Post Graduate Trainee, in onset and gradually progressed Nembian Raja Rajan B., independent mobility in both axes in intensity with radiation to right Junior Resident & Post Graduate Trainee, & didn’t fall forward on knee Sukanti Majhi, lower limb which was exacerbated Junior Resident & Post Graduate Trainee, elbow position. on walking and relieved on lying Alphonse Jithin, Junior Resident & Post Graduate Trainee, Examination of Musculoskeletal down with legs flexed at the knee Department of General Surgery, System and hip joints. She had low grade, SCB Medical College and Hospital, Cuttack. Odisha - 753 007. No Gibbus, no tenderness in intermittent type of fever with Specially Contributed to "The Antiseptic" spinal column, Fixed Flexion evening rise of temperature and Vol. 113 No. 10 & P : 26 - 28 26 THE ANTISEPTIC October 2016

Deformity of Hip joint about 15 nuclear palisading, well-formed provide a definitive diagnosis degrees. VEROCAY body and hyper cellular of retroperitoneal Schwannoma areas with edema. Hallmark before the operation. An ordinary Patient was admitted with pattern of Antoni A & Antoni B Schwannoma is depicted as a Differential Diagnosis of Psoas areas were identified and a final well-defined and inhomogeneous Abscess and Retroperitoneal tumor, diagnosis of INTRAMUSCULAR low-density mass on CT images 9 . lymphoma and was subsequently NEURILEMMOMA was On MRI, schwannomas are seen investigated. as masses with hypointensity established. [Figure-4] Routine hematological on T1-weighted images and Post operative recovery was investigations were within normal hyperintensity on T2-weighted normal and patient was discharged limits, ESR & ADA were normal images 10 . A definitive diagnosis is on post operative day 7 and in and the chest x ray and x ray based on pathological, histological, subsequent follow-ups there has spine revealed no abnormality. and immune histochemical been no evidence of recurrence. USG revealed findings consistent findings. Histologically, with PSOAS ABSCESS and USG Discussion schwannomas consist of compact guided Aspiration was done. 3oml cellular lesions (Antoni type A Schwannoma or neurilemmoma of clear fluid was aspirated that tissue) and loose, hypocellular is defined as a benign neoplasm coagulated immediately. The myxoid lesions with microcystic arising from the myelinated nerve coagulum was sent for analysis spaces (Antoni type B tissue). sheaths. It predominantly consists to rule out Hydatid disease, TB Characteristic histological finding of Schwann cells characterized & Malignancy. Microscopically is the presence of VEROCAY by their palisading architecture 5 . the cytosmear was pauci cellular BODY. Additionally, almost Schwannomas usually occur in the with a proteinaceous background all schwannomas show intense young and middle aged population and had no inflammatory cells immunohistochemical staining (20-50 yrs) with a female to male or parasites. Culture yielded no for S-100 protein, confirming preponderance of nearly 2:1 6 They growth. the neuroectodermal origin of the are neuroectodermal in origin tumor cells 11 . and most commonly occur in the P a t i e n t i m p r o v e d head and neck region (44.8% of symptomatically following Wide surgical resection in cases), upper limbs (19.1%), and aspiration and was discharged and cases of benign retroperitoneal lower limbs (13.5%). In contrast, followed up at monthly interval. schwannomas has been advocated On 3rd monthly checkup she had it is relatively rare in the retro by some authors based on their recurrence of symptoms and a peritoneum, with an occurrence belief that malignancy can never CT scan revealed [Figure-1] rate of 0.7%. Retroperitoneal be totally excluded. Others have Multiloculated cystic lesion in Rt region is a rare location for reported that, even in difficult lumbar region near Rt lower pole of schwannomas except in patients cases where complete removal kidney displacing it laterally with having Von Recklinghausen’s of the tumor was impossible and thin & thick septations & echoes disease. It is also noteworthy simple enucleation was performed within, without any evidence of to mention that malignant no tumor enlargement or malignant internal vascularity F/S/O Hydatid degeneration particularly takes change was observed. Wherever cyst. The patient was taken up place in association with Von possible complete surgical for Exploratory Laparotomy Recklinghausen’s disease. 7 excision must be performed for under General Anesthesia and the Symptomatology of benign schwannomas; since, they are lesion was approached through schwannomas is highly nonspecific not sensitive to radiotherapy and right lumbar incision. There was and depends on the location and chemotherapy. 12 The prognosis of an INTRAMUSCULAR (Psoas size of the lesion. If symptomatic, benign schwannomas is good and Muscle) Cystic lesion of size 8x4 the most frequent complication the most common presenting cm [Figure-2] which was excised is recurrence, probably due to features are vague abdominal in Toto and the cut sections incomplete excision, which is pain, heaviness and low backache. reported in only 5-10% cases 13 revealed solid and cystic areas Neurological symptoms though with multiple septations with areas have been described in literature Conclusion of hemorrhage. [Figure 3] but are usually rare 8 . Retroperitoneum is an Histopathology revealed There is no gold standard uncommon site for neurilemmoma fascicular arrangement of strand diagnostic method for and INTRAMUSCULAR spindle cells, alternatively hypo retroperitoneal Schwannoma; retroperitoneal neurilemmoma is cellular areas with marked therefore, it is difficult to a rarest of rare entity. Nonspecific THE ANTISEPTIC 27 October 2016