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CASE REPORT Table of Contents  
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Tumoral cutaneous rhinosporidiosis: Case report and review of literature


1 Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Burns and Plastic Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

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Date of Submission27-Jun-2021
Date of Decision07-Aug-2021
Date of Acceptance20-Nov-2021
Date of Web Publication26-May-2022
 

   Abstract 


Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi commonly affecting nasal mucosa, conjunctiva, and urethra. Subcutaneous tumor nodule presentation is rare and often mimics as sarcoma. Such tumoral rhinosporidiosis has been reported rarely. This report describes a 60-year male who presented with a solitary, firm, nontender swelling in posterior aspect of right leg with an ulcer and mimicking clinically as soft tissue sarcoma. Histopathology was diagnostic. Surgical excision was found to be useful.

Keywords: Rhinosporidiosis, sarcoma, tumor


How to cite this URL:
Nath N, Subba S, Giri SK, Pant P, Kanungo A, Mishra A, Mishra P. Tumoral cutaneous rhinosporidiosis: Case report and review of literature. Indian J Pathol Microbiol [Epub ahead of print] [cited 2022 Dec 9]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=345891





   Introduction Top


Rhinosporidiosis is a granulomatous disease caused by the protistan Rhinosporidium seeberi.[1] It is primarily a disease of the nasopharyngeal region, although primary and secondary dissemination to other parts of body has been reported.[2] It is endemic in India and Sri Lanka but has also been reported in the United States, South America, and Iran. In India, it is found more commonly in southern and central regions.[3] The disease is presumed to be transmitted through infected soil and water. Stagnant water is a potential source of infection as most infected patients are from rural background and have the habit of bathing in ponds. It is a chronic disease, with frequent recurrence after surgery, and occasional dissemination.[4] It usually involves nasal cavity but may rarely involve the lips, palate, uvula, maxillary antrum, epiglottis, larynx, trachea, bronchus, ear, scalp, vulva, vagina, penis, rectum, and the skin.[4],[5]

Cutaneous rhinosporidiosis mimicking clinically as soft tissue tumor is very rare. Only few such cases with tumoral presentations are reported in literature.[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18] Herein we report an HBsAg seropositive patient presenting with a solitary tumor-like swelling with a clinical diagnosis of sarcoma and histopathology confirmed the diagnosis of rhinosporidiosis and present a short review of all the cases presenting as soft tissue tumor reported till date.


   Case Report Top


A 60-year-old male from southern–eastern part of India, farmer by occupation known case of recurrent nasal rhinosporidiosis, presented with a painless swelling in the right calf. He had been operated twice before, for nasal rhinosporidiosis. On examination, he had a solitary 15 × 10 cm firm, nontender swelling in posterior aspect of right leg with an ulcer [Figure 1]a, which was bleeding on touch without any vascular or neurological compromise on the right leg. MRI of right leg showed a multicystic mass of 12.5 × 8 × 7 cm in the subcutaneous plane in posteromedial aspect of calf [Figure 1]b. He underwent complete excision of this mass. Per operatively, a well-encapsulated swelling was found in the subcutaneous plane was seen adhering to the medial head of gastrocnemius muscle [Figure 1]c, so a portion of muscle was excised along with the swelling. Postoperative follow-up shows a healed scar, and the patient is well without any recurrence 6 months post surgery.
Figure 1: 1a: Magnetic resonance imaging shows a multicystic mass of 12.5 × 8 × 7 cm in subcutaneous plane in posteromedial aspect of calf. 1b: Solitary 15 × 10 cm firm, nontender swelling in posterior aspect of right leg with an ulcer. 1c: Well encapsulated swelling was found in subcutaneous plane adhered to medial head of gastronemius muscle

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Pathologic findings

We received a skin attached large globular mass measuring 15.5 × 9.5 × 6 cm [Figure 2]a. Overlying skin measured 11 × 2.2 cm. An ulcero-nodular growth was noted on the skin measuring 5.5 × 4.5 × 1.4 cm. The external surface of the globular mass was grayish white to brown, smooth with nodular appearance. On cutting open, yellowish serous fluid was drained out. The cut surface was pale yellow, solid, and numerous cystic spaces filled with fluid. The cysts ranged in size from 1 cm to 6.5 cm [Figure 2]b.
Figure 2: 2a: Skin attached large globular mass measuring 15.5 × 9.5 × 6 cm with a skin nodule. 2b: Cut section of excised specimen showing tumorous mass with solid yellow areas and numerous cystic spaces. 2c: Section shows epidermis and the dermis filled with numerous sporangia. H and E,100×. 2d Section shows thick-walled sporangia with spores and numerous trophocytes in various stages of development. H and E,200×. 2e Mature sporangia with spores many of which have ruptured with the release of endospores. H and E,2. 2f: Grocott methanime silver highlighting the wall of mature sporangia

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Microscopic examination showed epidermis and the dermis showed numerous large thick-walled sporangia with plenty of endospores surrounded by a mixed inflammatory infiltrate comprising of lymphocytes and plasma cells [Figure 2]c. Some of the sporangia had released the spores eliciting a foreign body giant cell reaction [Figure 2]d. The sporangia were in different stages of maturation [Figure 2]e. Grocott methanamine silver stained the wall of the sporangiospores and the mature sporangia [Figure 2]f. A final diagnosis of rhinosporidiosis was rendered.

Discussion with review of literature

Rhinosporidiosis commonly involves the nose, nasopharynx, and palepebral conjunctiva. Less commonly involves oropharynx, larynx, genitalia, skin, viscera, and rare sites of involvement are the soft tissue and bone.

The common clinical presentation of rhinosporidiosis is nasal polyps or a conjuctival mass. Rarely, they present with multiple cutaneous nodules or as a soft tissue mass with bony involvement. Rhinosporidiosis presenting as a solitary tumor-like lesion is extremely rare and an extensive review of literature revealed 18 cases (including the present case) reported till date. In all these cases, there was a history of synchronous or metachronous mucosal rhinosporidiosis at some point of time. Avadhani et al.[11] reported rhinosporidiosis of the thigh swelling, who gave a history of having undergone excision of the nasal mass nearly 40 years back. In a similar report described by Aravindan et al.[5] reported rhinosporidiosis of the scapular mass in a patient who had a nasal polyp excised 20 years earlier. Hence the authors have proposed the term rhinosporidioma for such lesions. In an isolated case reported by Prabhakar et al.[14] there was no history of mucocutaneous or nasopharyngeal rhinosporidiosis and hence they considered it as primary cutaneous rhinosporidiosis.

Aravindan et al.[5] reported a 60-year-old man who was clinically and radiologically diagnosed as chondrosarcoma, however, histopathology yielded the correct diagnosis. Guru et al.[18] described in a 32-year-old man who was provisionally diagnosed as liposarcoma in a patient with a 12-cm swelling over posterior aspect of left mid-thigh for 6 months and an aspiration cytology rendered a diagnosis of rhinosporidiosis. Kar et al.[16] describe in his patient who is a 28-year-old farmer with swelling over his right shoulder and radiologically showed a lytic lesion involving the lateral end of the right clavicle, with a provisional diagnosis of primary bone tumor, a correct diagnosis was made by histopathological examination. Patients presenting with soft tissue masses or tumor-like lesion, fine needle aspiration cytology, or a histopathology examination is the gold standard for clinching a correct preoperative diagnosis.

A thorough literature search revealed seventeen cases (excluding the present case) who presented with tumoral cutaneous rhinosporidiosis showed a male preponderence (M: F-8:1) The average age of presentation is 43 years. One case was documented in a 11-year-old female child.[9] Lower extremity was commonly involved followed by upper extremity. The average size of the soft tissue lesion was 13 cm. Majority of the cases were clinically misdiagnosed as soft tissue sarcoma. The clinical features of all the reported cases are summarized in [Table 1]. As majority of cases had nasal mucosal lesions at some stage, we can assume that this tumor-like lesion is part of disseminated disease. Treatment of this disease has been essentially surgical excision on accessible sites. For disseminated disease, antimicrobial therapy is indicated along with dapsone. Our case was managed by surgical excision and on 6 months follow-up, no evidence of recurrence was noted.
Table 1: Summary of tumoral rhinosporidiosis cases reported till date

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Though aspiration cytology has its own limitations, it has still yielded the diagnosis in many of the cases, however, histopathology remains the gold standard for diagnosing such tumor-like lesions correctly, necessitating the correct management of the patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Chung KG, Bennett JE. Medical Mycology. London: Philadelphia, Lea & Febiger; 1992. p. 695-704.  Back to cited text no. 1
    
2.
Kumari R, Laxmisha C, Thappa DM. Disseminated cutaneous rhinosporidiosis. Dermatol Online J 2005;11:19.  Back to cited text no. 2
    
3.
Grover S. Rhinosporidiosis. J Ind Med Assoc 1975;64:93-5.  Back to cited text no. 3
    
4.
Arjun R, Khan F, Aravind B. Disseminated rhinosporidiosis masquerading as sarcoma. J Assoc Physicians India 2016;64:86-7.  Back to cited text no. 4
    
5.
Aravindan KP, Viswanathan MK, Jose L. Rhinosporidioma of bone. A case report. Indian J Pathol Microbiol 1989;32:312-3.  Back to cited text no. 5
    
6.
Date A, Ramakrishna B, Lee VN, Sundararaj GD. Tumoral rhinosporidiosis. Histopathology 1995;27:288-90.  Back to cited text no. 6
    
7.
Angunawela P, Tissera AD, Dissanaike AS. Rhinisporidiosis presenting with two soft tissue tumors followed by dissemination. Pathology 1999;31:57-8.  Back to cited text no. 7
    
8.
Houreih MA, Eyden B, Lucas SB, Roy S, Banerjee SS. Rhinosporidium seeberi: A case clinically mimicking soft tissue sarcoma. Histopathology 2006;49:208-10.  Back to cited text no. 8
    
9.
Raveenthiran V. Metastatic rhinosporidioma in a child. J Pediatr Surg 2006;41:853-5.  Back to cited text no. 9
    
10.
Tolat SN, Gokhale NR, Belgaumkar VA, Pradhan SN, Birud NR. Disseminated cutaneous rhinosporidiomas in an immunocompetent male. Indian J Dermatol Venereol Leprol 2007;73:343-5.  Back to cited text no. 10
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11.
Avadhani A, Pai K, Mohanty SP. Cutaneous rhinosporidiosis clinically masquerading as a soft tissue sarcoma- A rare occurrence. Int J Dermatol 2008;47:1153-4.  Back to cited text no. 11
    
12.
Mandal PK, Pandey A, Mukhopadhyay S, Roy S. Disseminated cutaneous rhinosporidiosis: A tumor like lesion with therapeutic challenge. Iran J Pathol 2014;9:277-80.  Back to cited text no. 12
    
13.
Prasad HL, Rao C, Girisha BS, Shetty V, Permi HS, Jayakumar M, et al. Subcutaneous rhinosporidiosis masquerading as soft tissue tumour: Diagnozed by fine needle aspiration cytology. Indian J Dermatol 2015;60:215.  Back to cited text no. 13
    
14.
Prabakar A, Karthikeyan G, Syed R. Rhinosporidioma: Primary cutaneous rhinosporidiosis-A rare case report. Indian J Case Rep 2016;2:27-9.  Back to cited text no. 14
    
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Agrawal P, Bhartiya R, Mehra R. Cutaneous rhinosporidiosis mimicking soft tissue tumour: A case report with review of literature. Ann Pathol Lab Med 2017;4:103-5.  Back to cited text no. 15
    
16.
Kar BK, Banchhor R, Thakur RP, Mohanty D, Monga N. Rhinosporidiosis with musculoskeletal manifestation: A case series. Indian J Orthop Surg 2018;4:405-10.  Back to cited text no. 16
    
17.
Barvadheesh RC, Dhandapani B, Gandhi RK. Soft tissue tumor leg-A rare presentation of cutaneous rhinosporidiosis. Surg Case Rep 2020;2:1-2.  Back to cited text no. 17
    
18.
Guru R, Rath BK. Rhinosporidiosis mimicking as soft-tissue tumour: A case report. Indian J Surg 2020;82:1273-5.  Back to cited text no. 18
    

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Correspondence Address:
Pritinanda Mishra,
Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha - 751 019
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_660_21



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